2016 - Endo Bridgeendobridge.org/arapca/cases_2016.pdf · 2016 6 20 - 23 October, 2016 Antalya - Turkey DATE TIME HALL SESSION TITLE 22.10 11.20-12.50 A Diabetes O-21 / The investigation

w w w . e n d o b r i d g e . o r g

Bridging the World of Endocrinology

SELECTED CASES

201620 - 23 October 2016

Cornelia Diamond Hotel, Antalya - Turkey

2016

220 - 23 October, 2016 Antalya - Turkey

SCIENTIFIC PROGRAM

Friday, 21 October 2016

08.40-09.00 Welcome and Introduction to EndoBridge 2016

MAIN HALL09.00-09.3009.30-10.00

10.00-10.3010.30-11.00

Chairs: Jens Bollerslev, Refik TanakolRecent advances in clinical and molecular understanding of metabolic bone disease - Nilgün Güvener Demirağ Approach to “drug holidays” “treatment failures” and possible combination therapy for postmenopausal osteoporosis Hasan AydınMedical therapy in primary hyperparathyroidism - Peter SchwarzUpdate on Vitamin D - Skeletal and nonskeletal effects - Ghada El-Hajj Fuleihan

11.00-11.20 COFFEE BREAK

11.20-12.50 HALL AHALL BHALL CHALL D

Interactive Case Discussion Sessions see page 5 for detailsBone / Calcium - Jens Bollerslev, Alper GürlekThyroid - Ralf Paschke, Seda SancakObesity / Lipids - Marc Cornier, Oğuzhan DeyneliMale Reproductive Endocrinology - Joshua Safer, Andrea Isidori

12.50-14.00 LUNCH


Interactive Case Discussion Sessions see page 5 for detailsBone / Calcium - Peter Schwarz, Ghada El-Hajj FuleihanThyroid - Michael McDermott , Georg BrabantObesity / Lipids - Marc Cornier, Sinem Küçüksaraç Kıyıcı Female Reproductive Endocrinology - Duarte Pignatelli, Füsun Saygılı


MAIN HALL15.50-16.2016.20-16.5016.50-17.2017.20-17.50

Chairs: Georg Brabant, M. Sait GönenPerplexing thyroid function tests - Michael McDermott Management of subclinical thyroid dysfunction including pregnancy - Georg Brabant Molecular diagnostic testing in evaluation of thyroid nodules - Ralf PaschkeDifferentiated thyroid cancer: Which type of surgery? How much RAI? How much suppression? - Ayşe Kubat Üzüm

2016


SCIENTIFIC PROGRAMSaturday, 22 October 2016

MAIN HALL09.00-09.3009.30-10.0010.00-10.3010.30-11.00

Chairs: Camilla Schalin-Jäntti, Bülent Yıldız Management of hyperglycemia and cardiovascular disease in type 2 diabetes - Rita Kalyani What is new in insulin therapy of diabetes? - Richard HoltDiabetes technologies: Current use and future perspectives Hans De VriesBariatric-metabolic surgery in type 2 diabetes - Kåre Birkeland



Interactive Case Discussion Sessions see page 6 for detailsDiabetes - Kåre Birkeland, Rita Kalyani Adrenal - Massimo Terzolo, Özlem ÇelikPituitary - AJ Van Der Lely, Nurperi GazioğluNeuroendocrine Tumors - Camilla Schalin-Jäntti, Eva Tiensuu Janson

12.50-14.00 LUNCH


Interactive Case Discussion Sessions see page6 for detailsDiabetes - Hans De Vries, Mohamed Hassanein Adrenal - Jerome Bertherat, Tevfik Demir Pituitary - Vera Popovic, John WassNeuroendocrine Tumors - Camilla Schalin-Jäntti, Eva Tiensuu Janson


MAIN HALL15.50-16.2016.20-16.5016.50-17.2017.20-17.50

Chairs: George Mastorakos, Fahri BayramThe “omics” of adrenocortical cancer - Jerome Bertherat Approach to adrenal incidentaloma: 2016 guidelines -Massimo TerzoloMale hypogonadism - Andrea IsidoriCare of transgender patients - Joshua Safer

2016


SCIENTIFIC PROGRAMSunday, 23 October 2016

MAIN HALL09.00-09.3009.30-10.0010.00-10.3010.30-11.00

Chairs: AJ Van der Lely, Melek Eda ErtörerEvaluation and management of sellar masses - Vera Popovic Can we really cure Cushing’s disease? - Susan Webb Medically refractory prolactinoma - John WassChallenges and controversies in diagnosis and management of acromegaly - Pınar Kadıoğlu


MAIN HALL11.20-11.50

11.50-12.20

12.20-12.5012.50-13.20

Chairs: Marja-Riitta Taskinen, İlhan YetkinAbdominal obesity, type 2 diabetes and cardiometabolic health - Amika Singh* Supported by International Chair on Cardiometabolic Risk

Ectopic fat: A new target for CVD risk management - Marja-Riitta Taskinen* Supported by International Chair on Cardiometabolic Risk

Food addiction: Does it really exist? - AJ Van der Lely The evolving science of nutrition and nutritional quality- Reyhan Nergiz Ünal

13.20-13.30 Closing comments and adjourn

2016


SELECTED CASESDATE TIME HALL SESSION TITLE

21.10 11.20-12.50 A Bone/Calcium O-01 / Postoperative Hypoparathyroidism: Treatment With Teriparatide

21.10 11.20-12.50 A Bone/Calcium O-02 / A case of primary hyperparathyroidism diagnosed during pregnancy

21.10 11.20-12.50 A Bone/CalciumO-03 / A striking finding during intraoperative jugular sampling in a case of parathyroid adenoma with low-normal serum intact parathyroid hormone levels

21.10 11.20-12.50 B Thyroid O-04 / A case of hypothyroidism requiring parenteral levothyroxine treatment

21.10 11.20-12.50 B Thyroid O-05 / Primary thyroid lymphoma presenting with subacute thyroiditis

21.10 11.20-12.50 B Thyroid O-06 / A rare case of thyrotoxic periodic paralysis with accompanying transient hyperglycemia

21.10 11.20-12.50 B Thyroid O-07 / A case with MEN-2b who has neurinoma of the tongue and eyes

21.10 11.20-12.50 B ThyroidO-08 / A case of papillary thyroid carcinoma in toxic adenoma: Are hyperfunctioning nodules truly innocent all the times?

21.10 11.20-12.50 B Thyroid O-09 / Medically refractory amiodarone-induced thyrotoxicosis: Two case reports

21.10 11.20-12.50 C Lipids/ObesityO-10 / Metabolic abnormalities associated with insulin resistance in a young patient with acquired partial lipodystrophy

21.10 14.00-15.30 A Bone/Calcium O-12 / Effect of zolendranate treatment on hearing loss in a patient with the Paget’s disease who has skull involvement

21.10 14.00-15.30 A Bone/Calcium O-13 / Thyroid nodule or parathyroid adenoma: a case report

21.10 14.00-15.30 B Thyroid O-14 / A case of thrombotic thrombocytopenic purpura in a toxic multinodular goiter patient on antithyroid medication

21.10 14.00-15.30 B ThyroidO-15 / Agressive follicular variant of papillary microcarcinoma presenting with extensive bone metastases and associated with weird thyroid function tests

21.10 14.00-15.30 B Thyroid O-16 / Thyrotoxic Periodic Paralysis

21.10 14.00-15.30 B Thyroid O-17 / Thyrotoxicosis with pleural effusion and hypoalbuminemia

21.10 14.00-15.30 B ThyroidO-18 / Plasmapheresis as a temporary management option in a patient with Graves’ disease and primary sclerosing cholangitis

21.10 14.00-15.30 B Thyroid O-19 / A lump on the scalp: An unusual presentation of papillary thyroid carcinoma

21.10 14.00-15.30 C Lipids/Obesity O-20 / Wernicke encephalopathy in a case who had gastric bypass surgery due to morbid obesity

2016


DATE TIME HALL SESSION TITLE

22.10 11.20-12.50 A Diabetes O-21 / The investigation for celiac disease in a newly diagnosed patient with diabetes is necessary in any case

22.10 11.20-12.50 A Diabetes O-22 / Mauriac syndrome still exists; a rare complication of type 1 diabetes mellitus

22.10 11.20-12.50 B AdrenalO-23 / Unilateral adrenalectomy improves cushing syndrome in a patient with primary bilateral macronodular adrenal hyperplasia

22.10 11.20-12.50 B Adrenal O-24 / CAH presenting as premature puberty with associated testicular adrenal rest tumors (TART)

22.10 11.20-12.50 B Adrenal O-25 / Different clinical presentations in three patients with adrenocortical cancer

22.10 11.20-12.50 B Adrenal O-26 / Bilateral cystic hemorrhagic adrenal incidentalomas

22.10 11.20-12.50 B AdrenalO-27 / Adrenocortical carcinoma presenting with Cushing’s syndrome in a patient with 8 years history of adrenal insufficiency and adrenomyolipomas

22.10 11.20-12.50 C Pituitary O-28 / Idiopathic granulomatous hypophysitis mimicking pituitary adenoma

22.10 11.20-12.50 C Pituitary O-29 / Case of giant prolactinoma in young man

22.10 11.20-12.50 C Pituitary O-30 / Pituitary stalk interruption syndrome: a rare cause of panhypopituitarism

22.10 11.20-12.50 D Neuroendocrine tumors

O-31 / How multifocal insulinomas can be diagnosed and treated in a case with MEN-1 disease?


O-32 / Catastrophic Cushing’s Syndrome: Report of a mortal case

22.10 14.00-15.30 A Diabetes O-33 / Effect of Imatinib on insulin therapy in a patient with Type 2 DM

22.10 14.00-15.30 B AdrenalO-35 / A Case Of Von Hippel-Lindau Disease with bilateral pheochromocytoma, abdominal paraganglioma and pancreatic neuroendocrine tumor

22.10 14.00-15.30 B Adrenal O-36 / Silent Pheochromocytoma in von Hippel-Lindau Disease: Report of a Case

22.10 14.00-15.30 B Adrenal O-37 / Adrenocortical carcinoma in pregnancy

22.10 14.00-15.30 B Adrenal O-38 / A case with adrenal cyst hydatid mimicking malignant tumour in MRI and PET-CT

22.10 14.00-15.30 C Pituitary O-39 / Hamartoma of hypothalmus presented as precocious puberty and epilepsy in 10-years old girl

22.10 14.00-15.30 C Pituitary O-40 / A rare cause of Diabetes Insipidus: Rosai Dorfman Disease


O-41 / Localization of an insulinoma by selective intraarterial calcium stimulation with hepatic venous sampling

22.10 14.00-15.30 D Neuroendocrine tumors O-42 / Known syndrome, a new case

2016


ORAL PRESENTATIONS

2016


O - 01 Bone/Calcium

POstOPerative HyPOParatHyrOidism: treatment WitH teriParatide

DENiz CAN GüVEN1, ÖMER ALPER GüRLEK2, SEDA OğUz2, NAfiyE HELVACI2

1Deparment of Internal meDIcIne, Hacettepe UnIversIty meDIcal facUlty, ankara, tUrkey 2Deparment of enDocrInology, Hacettepe UnIversIty meDIcal facUlty, ankara, tUrkey

Postoperative hypoparathyroidism is a common problem, follow-ing both thyroid and parathyroid surgeries. Conventionally vita-min D and elementary calcium have been the mainstay of therapy. Herein, we present our experience with teriparatide (recombinant parathormone) in a postoperative hypoparathyroidism patient. A 28-year-old female patient was referred to our clinic due to euthy-roid autoimmune thyroiditis with thyroid nodules. The patient was followed without treatment. Three years later, the patient applied to our clinic with inflammatory low back pain and morning stiffness. An MRI of sacroiliac joint showed bilateral grade III sacroileitis. Af-ter the patient’s evaluation with these findings, the diagnosis was made as ankylosing spondylitis. Before starting the steroid treat-ment, a bone mineral density measurement was performed and showed a lumbar spine z score of -6.6 and femur z score of -4. To-tal calcium and phosphate levels were 10.5 mg/dL and 2.5 mg/dL, respectively. Serum PTH test was ordered and it was 1619 pg/ml. Parathyroid ultrasonography revealed two lesions located inferior of each thyroid lobe which were compatible with parathyroid gland pathology. Total thyroidectomy and parathyroid gland excision and servical lymph node dissection were performed with the prelimi-nary diagnosis of parathyroid carcinoma. Histopathological exam-ination of surgical specimen showed chronic lymphocytic thyroiditis and parathyroid adenoma. In the postoperative period, the patient’s total calcium levels was around 5.5-6 mg/dL. IV calcium replace-

ment with oral calcitriol and calcium carbonate was required for three weeks postoperatively. After discharge with a maintenance regimen of calcitriol 12 mcg/day, calcium carbonate 15 grams/day and cholecalciferol 3600 IU/day, the patient’s hypocalcemia persist-ed and hyperphosphatemia developed. At this point we decided to start subcutaneous recombinant parathyroid hormone. Teriparati-de (20 μg/12 h s.c.) was started. This therapy enabled dose reduc-tion of calcitriol down to 4.5 mcg/day and calcium carbonate to 9 grams/day. Hyperphosphatemia was resolved. After seven months of therapy teriparatide dose was tapered to a daily single dose which was ceased completely after another month. Hypocalcemia did not recur after the cessation of teriparatide. During 5 years of follow up, the patient has been normocalcemic under the reg-imen of calcitriol 1.5 mcg/day and calcium carbonate 3 grams/day. Short term use of teriparatide is reported in multiple case reports especially in patients with thyroidectomy and neck dissection. A prospective study with four years follow up provided data for the safety and efficacy of long term teriparatide treatment in postop-erative hypoparathyroidism. Although further studies are required for long term effects, teriparatide seems to be a good choice with a favorable safety profile for refractory postoperative hypoparathy-roidism.

Keywords: refractory postoperative hypoparathyroidism, teri-paratide, parathyroid adenoma

2016


Primary hyperparathyroidism (PHPT) is a rare disorder. Incidence is 8/100000.This incidence is same among child bearing age wom-en and pregnants. PHPT during pregnancy is associated with sev-eral risks to the mother and fetus especially when maternal total serum calcium is >11 mg/dL. In gestational PHPT cause is a single adenoma in %80-85 patients, hyperplasia in %10-12 patients, mul-tiple adenoma in %2 patients and carcinoma %1 patients as gen-eral population. We present a case of PHPT diagnosed during third trimester of pregnancy.

A 43 years old woman, was under follow up of obstetric clinic be-cause of her fourth pregnancy. She was admitted to the hospital with abdominal pain, nausea and constipation at 27th week of ges-tational age. Complete blood count and biochemical tests were to-tally normal except hypercalcemia [Ca:11,4 mg/dl (8,7-10,4)]. She was referred to our clinic because of hypercalcemia. There were no pathological findings on her physical examination. Hyperpara-thyroidism was detected with initial tests for differential diagno-sis; creatinine:0,62 mg/dl (0,5-11), Ca: 11,4 mg /dl (8,7-10,4),P: 2,67 mg/dl(2,4-5,5), PTH: 140 pg/ml (14-72),25-OH D3:18,2 ng/ml (30-100), 24 hour urine Ca:310 mg/24h (<300). An adenoma like hypoechoic lesion (image1) with size of 19 X 6 X 15 mm was de-tected with ultrasound on right inferior thyroid region. We didn’t plan surgery because she was in the third trimester of pregnancy. We planned close follow up during pregnancy. Hospitalization was

required several times because of hypercalcemia during pregnancy and normocalcemia was enabled with intraveneous saline infusion. She gave birth to a hypotonic and hypoglycemic male infant at 37th week of gestatonal age with caesarean section. Baby’s serum cal-cium level was 10,5 mg /dl and PTH level was 1 pg/ml after birth. Mother’s serum calcium level was 11.6 before labor and normo-calcemic state was also enabled with intraveneous saline infusion. Her serum calcium level is 10,2 mg/dl at postpartum follow up. We planned surgical operation later on.

Keywords: pregnancy, hyperparathyroidism, pregnancy and hyper-parathyroidism, hypercalcemia and pregnancy

figure 1. ultrasound view of parathyroid adenoma

O - 02 Bone/Calcium

a Case Of Primary HyPerParatHyrOidism diagnOsed during PregnanCy

HAKAN DOğRUEL, NUSRET yILMAz, ÖzLEM DOğAN, HüMEyRA BOzOğLAN, RAMAzAN SARI, HASAN ALi ALTUNBAş, MUSTAfA KEMAL BALCI

akDenIz UnIversIty HospItal, antalya, tUrkey

2016


Aim: Primary hyperparathyroidism (PHP) is the most common cause of hypercalcemia based on outpatient clinic. The diagnosis of PHP is generally straightforward with high intact PTH (iPTH) and hypercalcemia. But, PHP does not always exhibit those biochemi-cal features. Less known phenotype of PHP is the hypercalcemia with normal level of iPTH. We report a case of parathyroid adenoma with low normal peripheral iPTH and high jugular PTH levels pre-senting as hypercalcemia.

Case: A thirty-six year old woman who have type 2 diabetes melli-tus was admitted to the hospital with the diagnosis of acute pancre-atitis. She had no cholelithiasis. Serum amylase and lipase levels were high. Acute edematous pancreatitis was shown on comput-ed tomography. She had hypertriglyceridemia in her past medical history. Serum of the patient was observed as lipemic. Hypertri-glyceridemia could be a reason for the pancreatitis. On laboratory examination at admission to the hospital; calcium level was 8.0 mg/dL (8.8-10.2), intact PTH (iPTH) level was 28.8 pg/mL (12-88) and 25-hydroxy vitamin D level was 6.5 ng/mL. Cholecalciferol was giv-en intramuscularly as 300.000 IU. In her past medical history, hypo-thyrodism was present. Thyroid ultrasonography was performed. There was a hypoechoic solid nodule on right thyroid lobe sized as 7x3mm. There was also 17x6 mm sized lesion at the lower part of right thyroid lobe that revealed as possible parathyroid adenoma. On Tc 99m MIBI scintigraphy, a lesion was seen at the lower part of

right thyroid lobe (figure 1). This lesion was thought as parathyroid adenoma. There was no osteoporosis according to bone mineral densitometry. After replacement of vitamin D and acute pancreati-tis subsided, serum Ca and iPTH levels were as follows: 10.6, 10.7, 10.9 mg/dL and 28.8, 33.3, 31.7 pg/mL, respectively. Twenty-four hour calcium excretion was 403 mg/day, serum phosphorus was 2.3 mg/dL. iPTH measurement was repeated in another accredited laboratory and was found as 27 pg/mL. iPTH was measured also by using dilution technique and obtained as 38 pg/mL. Hydration and furosemide treatment were performed, but Ca level was still high, which was 11.4 mg/dL. Besides hypertriglyceridemia, we thought that hypercalcemia could precipitate the episodes of pancreatitis. Based on the findings of hypercalcemia, hypercalciuria, positive so-nographic and scintigraphic images, operation was performed with the possible diagnosis of PHP. PTH measurement was taken from the right jugular vein. Intraoperative PTH was 634 pg/mL. Histo-pathological examination revealed as parathyroid adenoma. Post-operative hypocalcemia didn’t occur and postoperative calcium and iPTH levels were normal.

Conclusion: Clinicians should be aware of the presence of low-nor-mal iPTH in PHP. If the clinical suspicion of PHP is high as in our case, surgery should be performed after exclusion of other causes of hypercalcemia.

Keywords: Hypercalcemia, low-normal PTH, parathyroid adenoma

O - 03 Bone/Calcium

a striking finding during intraOPerative jugular samPling in a Case Of ParatHyrOid

adenOma WitH lOW-nOrmal serum intaCt ParatHyrOid HOrmOne levels

ILGIN yILDIRIM şiMşiR1, BANU şARER yüREKLi1, HATiCE ÖzIşIK1, MURAT ÖzDEMiR2, yEşiM ERTAN3, ÖzER MALAy2, GÖKHAN ÖzGEN1

1 ege UnIversIty facUlty of meDIcIne, Department of enDocrInology, IzmIr, tUrkey 2 ege UnIversIty facUlty of meDIcIne, Department of general sUrgery, IzmIr, tUrkey

3 ege UnIversIty facUlty of meDIcIne, Department of patHology, IzmIr, tUrkey

2016


Introduction: Some hypothyroid patients need high doses of oral levothyroxine (LT4) due to poor adherence to treatment, not taking the drug on an empty stomach, malabsorption, and co-administration of drugs interfering with absorption. We present a case of isolated mal-absorption of LT4 responding to intramuscular (IM) therapy.

Clinical Case: A 32 year old female patient was admitted to hospital due to hair loss and massive weight gain in 2008. She had thyroidec-tomy and radioactive iodine therapy in 2004. She was on 400 μg/d LT4 therapy. Physical examination revealed dry skin, non-pitting oedema, and diminished deep tendon reflexes. History of systemic diseases, diarrhea, and co-administration of any other drugs or herbal products interfering with LT4 absorption were absent. Thyroid function tests (TfT) did not improve despite medication under supervision.

Gastrointestinal endoscopy and stool analysis was normal. Antipari-etal, endomysium, and transglutaminase antibodies were negative. Triiodothyronine added to high-dose LT4 therapy was ineffective.

To evaluate LT4 malabsorption, 1000 µg LT4 was administered orally and TfT were measured with 2 hour intervals for 6 hours. fT4 level did not change. During the test she developed aphasia, loss of vision, and hemiparesis. Cranial imaging was normal and transient ischemic attack (TIA) was diagnosed. The neurologic symptoms regressed with anti-oedema and acetylsalicylic acid (ASA) therapy. She was discharged with daily 450 µg LT4 and 75 µg triiodothyronine therapy.

She was lost to follow-up until 2012. During this period she had another TIA during intravenous (IV) therapy in another center. fur-thermore rectal route could not be tolerated and vaginal route was unsuccessful.

Her treatment schedule was planned as weekly increased doses of 200, 500, 1000, and 1200 µg IM LT4. TfT were measured at weekly in-tervals. She developed left sided loss of strength during 1200 µg dose. Cranial imaging was again normal. The symptoms regressed within a week with ASA therapy. Detailed tests for coagulopathy were normal.

TfT quickly normalized with IM 500 µg LT4 twice a week. No other TIAs occurred.

After euthyroidism was achieved, parenteral route was switched to oral route according to the assumption that malabsorption due to oedema in the gastrointestinal tract secondary to hypothyroidism might have improved. However hypothyroidism ensued soon. Pre-vious regime was started again. She was euthyroid at the last time she was seen.

Conclusion: Impaired LT4 absorption may continue despite vigorous acts against factors interfering with absorption. Due to its limited mar-keting and its high cost, LT4 is administered for short term via slow IV infusion. The only risk for TIA and atherosclerosis was prolonged hypothyroidism. She did not experience acute variations in serum T4 during high dose LT4 therapy to explain to TIAs. An adverse effect of high dose LT4 therapy seems to be the best explanation for TIAs.

Keywords: hypothyroidism, malabsorption, parenteral levothyrox-ine, transient ischemic attack

Table 1. The changes in the levels of free throxine and thyroid stimu-lating hormone after administration of intramuscular 500 µg LT4 twice a week (fT4: free thyroxine, TSH:Thyroid stimulating hormone)

O - 04 thyroid

a Case Of HyPOtHyrOidism requiring Parenteral levOtHyrOxine treatment

HANDE PEyNiRCi1, BENGüR TAşKIRAN2, ERDiNç ERTüRK3, PINAR şişMAN3, CANAN ERSOy3

1 kastamonU state HospItal, kastamonU, tUrkey 2 yUnUs emre state HospItal , eskIşeHIr, tUrkey

3 UlUDag UnIversIty , bUrsa, tUrkey

2016


Table 2. The changes in the levels of free T4 and TSH after administration of oral 1000 mcg LT4 at the end of a 6-hour monitoring

TFT baseline 2nd hour 4th hour 6 th hour

fT4 (ng/dL) 0.40 0.40 0.40 0.40

TSH (μIU/mL) 100 81.81 84.74 93.48

2016


Background: Primary thyroid lymphoma (PTL) is defined as a lympho-ma involving only the thyroid gland or the thyroid gland and adjacent (regional) neck lymph nodes. Thyroid lymphoma represents 4% of all malignancies that is a relatively rare disease often posing a diagnostic challenge (1). Reaching the final diagnosis can be delayed if insufficient biopsy material is obtained. fNA diagnosis of thyroid lymphoma, par-ticularly due to the histological similarities with thyroiditis and the high coincidence of these pathologies within the same gland, which results in increased false-negative rates from sampling error (2). The present study describes the case of a patient who was treated as subacute thy-roiditis and diagnosed as having diffuse large B-cell lymphoma.

Clinical Case: A 61-year-old female presented with anterior neck pain, hoarseness and rapidly expanding mass of the neck diagnosed as subacute thyroiditis and she had recieved NSAi at least 2 months for her pain 4 months earlier. fine needle aspiration cytodiagnosis was benign. The patient was admitted to our hospital because of in-creasing dyspnea. Laboratory studies revealed an euthyroid state; antithyroglobulin and thyroid peroxidase antibodies were both pos-itive. In PA chest X-ray trachea was narrowed and shifted to the left side. A neck ultrasound showed a significantly enlarged, diffuse pa-renchymal inhomogeneity and ill defined border over right lobe and isthmus of the thyroid gland. Also, the trachea was narrowed and pressed to the right. Tru-cut biopsy was performed and histological diagnosis was Diffuse large B-cell lymphoma of the thyroid gland. A computed tomography scan demonstrated that the trachea was markedly compressed and displaced by the mass. His respiratory status was so urgent that the patient was given corticosteroids and saved her from receiving a tracheostomy. After she recieved che-motherapy she was in a good physical condition.

Conclusion: The most common presentation of subacute thyroid-itis is an anterior neck pain radiating up to the jaw and ear. Other causes of pain in the thyroid gland should be taken into consider-ation during differential diagnosis, especially when a patient pres-ents with misleading symptoms like progressive dyspnea, dyspha-gia or dysphonia. Such a presentation should be acknowledged and this leads to early diagnosis, treatment and prevention of unneces-sary surgery.

Keywords: Primary thyroid lymphoma, subacute thyroiditis, neck mass

figure 1: A computed tomography

figure 1. A computed tomography the trachea was markedly compressed and displaced by the mass.

figure 2: PA chest X-ray

figure 2. PA chest X-ray yazılacak narrowing and shifting of trachea

O - 05 thyroid

Primary tHyrOid lymPHOma Presenting WitH suBaCute tHyrOiditis

SEVGüL fAKI1, NESLiHAN çUHACI1, OyA TOPALOğLU1, CüNEyT BiLGiNER1, BERNA ÖğMEN1, HAKAN KORKMAz2, üNSAL HAN3, REyHAN ERSOy1, BEKiR çAKIR1

1 Department of enDocrInology anD metabolIsm, ankara yIlDIrIm beyazIt UnIversIty, scHool of meDIcIne, ankara, tUrkey

2 Department of otorHInolaryngology, ankara yIlDIrIm beyazIt UnIversIty, scHool of meDIcIne, ankara, tUrkey 3 Department of patHology, ankara yIlDIrIm beyazIt eDUcatIon anD researcH HospItal, ankara, tUrkey

2016


Introduction:Thyrotoxic periodic paralysis (TPP) is an uncommon complication of thyrotoxicosis which is associated with paralysis and hypokalemia. Treatment of TPP consists of adequate potassi-um replacement, beta-adrenergic blockage and control of hyper-thyroidism.

Clinical Case: A-51-year-old male patient was admitted to emer-gency department with complaint of muscle weakness which start-ed 2 weeks ago but was aggravated a high carbohydrate intake. He was admitted to department of cardiology in another hospital 3 months ago because of dyspnea and his coronary angiography showed normal coronaries. He had no history of diabetes or hyper-tension. One week after coronary angiography he was applied to endocrinology and metabolism unit with complaint of sweating and subclinical hyperthyroidism was diagnosed. Non-iodized salt was recommended. In his admission to emergency department in our hospital; his physical examination revealed proximal muscle weak-ness in both upper extremities (4-/5) and lower extremities (2/5). His electrocardiogram showed u waves with 90 beats per minute. Other physical examination findings were unremarkable. His bio-chemical analysis results were as follows: random plasma glucose: 363mg/dl, serum creatinine: 0.53mg/dL, sodium: 138mmol/L, potassium: 1.9mmol/L, total calcium: 9.13mg/dL, phosphorus: 1.17mg/dL, albumin 4.22g/dL, Hb: 13.0g/dL. His white blood cell count and thrombocyte levels as well as liver function tests were normal. Despite high glucose levels, he had neither acidosis nor

ketosis. He had overt hyperthyroidism with TSH: <0.05μIU/mL and free T4: 3.47ng/dL. His thyroid receptor antibody level was found to be positive (5.3U/L) as well as anti thyroid peroxidase antibody and anti-thyroglobulin antibody levels. Thyroid ultrasonography was consistent with thyroiditis. He was diagnosed as Graves’ disease and TPP. Intravenous potassium supplementation was performed with close monitoring and peroral 20mg propranolol as well as 30mg methimazole was given to the patient. His blood glucose lev-els were high being in the range of 250-400 mg/dl and his HbA1c level was 4.7%. Basal-bolus insulin treatment was given during his hospital stay and normoglycemia was achieved with insulin treatment. Also his blood pressure was measured to be >140/90 mmHg during his stay and 10mg amlodipine was prescribed. After normalization of potassium levels he didn’t have any complaints of muscle weakness. His insulin requirement decreased during his stay and he was discharged with 10 units of insulin glargine and 10mg amlodipine as well as 20mg propronolol and 30mg methi-mazole. He was invited to consultation 1 week after his discharge and his free T4 level was 1.27ng/dL and he did not have any need of insulin, amlodipine and propronolol.

Conclusion: TPP is an uncommon but potentially fatal complica-tion of hyperthyroidism. Despite being an easily treatable condition, it could have life-threatening consequences if it is overlooked.

Keywords: Thyrotoxicosis, Transient hypokalemic paralysis, Graves’ disease

0 - 06 thyroid

a rare Case Of tHyrOtOxiC PeriOdiC Paralysis WitH aCCOmPanying transient HyPerglyCemia

BAşAK ÖzGEN SAyDAM, üMiT çAVDAR, BARIş AKINCI, TEVfiK DEMiRDepartment of enDocrInology anD metabolIsm, DokUz eylUl UnIversIty facUlty of meDIcIne, IzmIr, tUrkey

2016


Introduction: Multiple endocrine neoplasia type 2b (MEN 2b) is a rare and dominantly inherited syndrome characterized by medul-lary thyroid cancer, pheochromocytoma and mucosal neuromas. The most characteristic feature of MEN2b is multiple mucosal neuromas seen in almost all cases. As it is rarely seen, we have decided to present this case.

Case: A male patient aged 41 underwent bilateral total thyroidecto-my operation due to multinodular goiter in 2004 and the patholog-ical examination showed that the patient developed medullary thy-roid carcinoma. In surveillance, upon the detection of pathological lymph nodes in the neck, left neck dissection was applied.

In 2005, adjuvant radiotherapy was applied to the neck. In 2006, in genetical analysis, M918T heterozygous mutation was detected in RET gene. In 2008, as a mass was identified in the left adrenal gland, laparoscopic left adrenalectomy was applied. The pathology was determined as pheochromocytoma. Calcitonin value had not been checked in the preoperative period. Calcitonin value was 120 pg/mL in the post-operative period.

In the family history of the patient, his elder brother had had an operation due to pheochromocytoma.

In his physical examination, there were neuromas on the tongue and eye lids (Picture 1). He had hyperpigmented areas on his left shoulder (Picture 2)

The latest value for calcitonin was 88.6 pg/mL and CEA was 6,78 ng/mL. 24 hour urine metanephrine and normetanephrine levels were normal.

In laboratory examination, TSH was 2.26µIU/ mL and fT4 was 1.37 ng/dL. He was euthyroid with 150 mcg/day L-thyroxine treatment. The patient has been surveilled through the ultrasonography of neck (USG) and 24 hour urine metanephrine and normetanephrine.

Conclusion: The medullary thyroid carcinoma can progress most agressively in MEN2b. In this syndrome metastasis were defined even in the first year of life.

Mutations at codon 883 and 918 mutations with MEN 2b are at in-creased risk of agressiveness. In our case,codon 918 mutation was also detected.

It is recommended that the family members of these cases are screened for medullary thyroid carcinoma and if there is any carry-ing these mutations, they should be treated with prophylactic total thyroidectomy. for these cases, lifelong surveillance is required.

Keywords: Multiple endocrine neoplasia type 2b, medullary thy-roid cancer, mucosal neuromas

Picture 1

Picture 1. Neuromas on the tongue

Picture 2. hyperpigmented areas on the left shoulder

O - 07 thyroid

a Case WitH men-2B WHO Has neurinOma Of tHe tOngue and eyes

HATiCE ÖzIşIK1, BANU şARER yüREKLi1, NiLüfER ÖzDEMiR KUTBAy1, MAHiR AKyILDIz2, GÖKHAN ÖzGEN1

1 Department of enDocrInology, ege UnIversIty, İzmIr, tUrkey 2 Department of general sUrgery, ege UnIversIty, İzmIr, tUrkey

2016


Introduction: Thyroid scintigraphy should be performed for the evaluation of thyroid nodules in case of suppressed Thyroid Stim-ulating Hormone (TSH) level. While the malignancy probability of hypofunctioning nodules is 15%, this ratio is generally lower than 1% in hyperfunctioning nodules. So we would like to present a case of toxic adenoma with the diagnosis of papillary thyroid carcinoma.

Case: forty-four-year-old woman patient had presented with the diagnosis of thyroid nodule. Thyroid fine needle aspiration biopsy (TfNAB) was performed for the evaluation of thyroid nodule mea-sured as 47x12 mm. This nodule was in mixed solid form bear-ing cystic components. TfNAB revealed that the thyroid nodule was benign. Propylthiouracil had been started before admission to our clinic according to laboratory values of TSH and free thy-roxin (fT4) which were 0.005 µIU/ mL and 1.75 ng/dL, respective-ly. On admission to our endocrinology clinic we performed thyroid scintigraphy showing hyperfunctioning thyroid nodule with scene of suppressed rest of thyroid region. Thyroid auto-antibodies were negative. Radioactive iodine therapy was recommended as a mo-dality of treatment. However, the patient preferred surgery for her

treatment with the concern of her father’s death due to leukemia. Our ultrasonographic evaluation was consistent with a thyroid nodule located at right lobe with a diameter of 43x18x28 mm. The nodule was mixed type solid nodule with regular borders and thin hypoechoic peripheral halo. Central chaotic hypervascularity and microcalcification were not detected. The patient underwent right thyroid lobectomy. Pathology report revealed macrofollicular vari-ant of papillary thyroid carcinoma. The tumor was 3.5 cm in diam-eter with regular border and 0.2 cm away from the surgical border. The tumor didn’t spread out of the thyroid capsule. Second thyroid surgery was performed due to remaining thyroid tissue. After that ablative radioactive iodine therapy was applied.

Conclusion: Although malignant potential of hyperfunctioning thyroid nodules is low, possibility of thyroid carcinoma cannot be excluded in those thyroid nodules. So, hyperfunctioning thyroid nodules warrants careful evaluation and appropriate therapy. We wanted to draw attention of the clinicians for this rare issue.

Keywords: toxic adenoma, papillary thyroid carcinoma, surgery

O – 08 thyroid

a Case Of PaPillary tHyrOid CarCinOma in tOxiC adenOma: are HyPerfunCtiOning nOdules truly innOCent all tHe times?

BANU şARER yüREKLi1, HATiCE ÖzIşIK1, NiLüfER ÖzDEMiR KUTBAy1, ÖzER MAKAy2, GÖKHAN ÖzGEN1, AyşEGUL AKGüN3

1 ege UnIversIty facUlty of meDIcIne, enDocrInology Department, IzmIr, tUrkey 2 ege UnIversIty facUlty of meDIcIne, general sUrgery Department , IzmIr, tUrkey 3 ege UnIversIty facUlty of meDIcIne, nUclear meDIcIne Department , IzmIr, tUrkey

2016


Background: Amiodarone is the most commonly prescribed an-tiarrhythmic drug that is used mainly for the treatment of atrial fibrillation. Amiodarone-induced thyrotoxicosis (AIT) is a therapeu-tic challenge and it may be due to iodine-induced excessive syn-thesis of thyroid hormone (Type 1) or a destructive thyroiditis (Type 2). Herein we present two cases of medically refractory AIT treated with a surgical approach.

Case 1: A 24-year-old female with recurrent ventricular fibrillation attacks due to cardiomyopathy was referred to our clinic because of thyrotoxicosis. She had an intracardiac defibrillator (ICD), ejection fraction (Ef) was 35%, and had been using amiodarone orally for three months. Thyroid function test (TfT) results revealed thyro-toxicosis. Thyroid gland size was normal but mildly parenchymal heterogeneity and decreased vascularization were detected. Car-diology department discontinued the amiodarone therapy. Due to diagnosis of type 2 AIT, steroid therapy was started. In addition to increased steroid dosage, 3 weeks later methimazole and lithium therapies were initiated because of the progression in clinical and laboratory findings. Despite current medical therapies, no improve-ment was detected. Patient was diagnosed as non-responsive type 2 AIT and surgical approach was decided. In order to regulate the TfT levels, 12 sessions of plasmapheresis were performed. Thyroid function tests returned to normal. Subsequently, total thyroidecto-my was performed without perioperative complication. Patient was discharged from hospital by the third day.

Case 2: A 39-year-old male with history of hypertrophic cardiomy-opathy due to ventricular arrhythmia, heart failure (Ef 20%) and ICD was hospitalized to the coronary intensive care unit. Amiodarone infusion therapy was started. While amiodarone therapy continued thyrotoxicosis was detected and patient was consulted to endocri-nology clinic. The thyroid gland was normal but the vascularization was reduced. Thyroid scintigraphy showed active involvement on thyroid gland. Due to diagnosis of Type 1 AIT, antithyroid therapy was initiated while streoid therapy was added a week later. The therapy was continued in high doses but no response was seen and arrhythmias continued. Because of high-risk for thyroid surgery firstly 30 mCi radioiodine was administered but thyrotoxicosis con-tinued. Therefore, total thyroidectomy was performed under strict cardiology consultation. No perioperative complications were seen. Levothyroxine replacement started and tapering of steroid therapy was planned. Patient was discharged from hospital by the fourth day.

Discussion: Amiodarone-induced thyrotoxicosis is a diagnos-tic challenge due to its complicated pathogenesis and unreliable response to therapy. After the classification of AIT, the appropri-ate treatment must be started immediately. Persistent treatment choices such as surgery should be considered in uncontrolled pa-

tients or patients that are refractory to medical treatment.

Keywords: Amiodarone, thyrotoxicosis, resistant

O – 09 thyroid

mediCally refraCtOry amiOdarOne-induCed tHyrOtOxiCOsis: tWO Case rePOrts

NARiN NASIROğLU iMGA1, zEyNEP çETiN1, ALPER çAğRI KARCI1, DiLEK BERKER1, SERDAR GüLER2

1 Department of enDocrInology, ankara nUmUne eDUcatIon anD researcH HospItal, ankara, tUrkey 2 Department of enDocrInology, HItIt UnIversIty facUlty of meDIcIne, corUm, tUrkey

2016


Introduction: About 250 patients with acquired partial lipodystro-phy (APL) have been reported so far. It is characterized by the loss of adipose tissue from the face, arms and upper part of the body. The disease usually starts during childhood or adolescence. fat loss often come into view in months or years. females are more fre-quently affected. Although the etiology of APL is unclear, increased complement activity has been reported in some APL patients.

Case: A 23-year-old female reported fat loss on her face that she first noticed when she was 12. She was diagnosed with diabetes when she was 21 which was treated with metformin. On physical examination, fat loss was remarkable on her face, arms and up-per trunk (fig.1a). Acanthosis nigricans was noticed on her arm-pits and around her neck (fig.1b). Her laboratory results were as follows: fasting blood glucose: 111 mg/dl, HbA1c: 6.1%, fasting in-sulin: 34.3 ulU/ml, AST: 40 U/L, ALT: 65 U/L, triglyceride: 393 mg/dl, HDL-cholesterol: 32 mg/dl, C3: 1.02 g/L (0.9-1.8), C4:0.29 g/L (0.1-0.4), and microalbumin in spot urine: 26.5 mg/L. Abdominal ultrasonography showed grade 1 hepatosteatosis. Echocardiogra-phy was normal. No Drusen-like lesion was detected in eye exam-ination.

Conclusion: Although previous studies reported that metabolic ab-normalities were rarely observed in APL, our recent study showed that metabolic abnormalities associated with insulin resistance are more common than previously thought. Our observation on this newly diagnosed APL patient also supports the idea that a regular screening for metabolic abnormalities should be carried out in pa-tients with APL even at young age.

Keywords: Acquired partial lipodystrophy, metabolic abnormali-ties,Drusen-like lesion

figure 1a, 1b: A case of acquired partial lipodsyttrophy (APL)

O – 10 lipids/Obesity

metaBOliC aBnOrmalities assOCiated WitH insulin resistanCe in a yOung Patient WitH

aCquired Partial liPOdystrOPHyNiLüfER ÖzDEMiR KUTBAy1, BANU SARER yüREKLi2, HALiT DiRi1, HATiCE ÖzIşIK2, zEKi yAşAR3,

füSUN SAyGILI2, BARIş AKINCI4

1 gazI yasargIl traInIng anD researcH HospItal, DIvIsIon of enDocrInology, DIyarbakIr, tUrkey 2 ege UnIversIty, DIvIsIon of enDocrInology, IzmIr, tUrkey

3 selaHaDDIn eyyUbI state HospItal, DIvIsIon of plastIc sUrgery, DIyarbakIr, tUrkey 4 DokUz eylUl UnIversIty, DIvIsIon of enDocrInology, IzmIr, tUrkey

2016


Background: Paget’s disease is a localised disorder of bone re-modelling characterized by the excessive resorption and new bone formation. Clinical features of Paget’s disease are pain, fractures, deformity, and manifestations of the neurologic, rheumatologic, or metabolic complication of the disease. However, at least two-thirds of patients are asymptomatic.

Case: We reported a 50 years old woman attend neurosurgery outpa-tient clinic complaining of headache for about a year. The patient was reffered to endocrinology outpatient clinic because the cranial mag-netic resonance imaging (MRI) of the patient raised suspicion of Pag-et’s disease of bone. from the anamnesis of the patient we learned that she also has progressive hearing loss. On physical examination, she has enlargement of the skull with left frontal bossing.

Laboratory findings; serum calcium, phosphorus, urea, creatinine, liver enzyme levels were normal. Serum alkaline phosphatase (ALP) activity and parathyroid hormone (PTH) levels were increased and serum vitamin D level was deficient.

Imaging studies; Cranial MRI of the head show marked thickening of the diplopic space, diffuse calvarial thickening. Left side is more thick than the right side. There are heterogenous signal differences in all sequences. Bone scan releaved intense uptake of the radionuclide in the calvarium that is generally diffuse but heterogenous only in some parts and more marked in the left fronto-parietal bone. There is also intense uptake of the radionuclide on the left femur.

The patient was sent to ear specialist for evaulation of her hearing loss. Physical examination of her ears were normal. In her audio-metric assesment there was bilateral asymmetric sensorineural hearing loss in pure tone audiometry of patient. Pure tone averages of air/bone conduction were 25 dB/25 dB on the right side and 35 dB/27 dB on left side.

After vitamin D deficiency was corrected serum PTH level became normal but serum ALP level was still high. A single 5 mg dose iv zolendronate was given for the treatment of her Paget’s disease. Six months following this treatment serum ALP activity, calcium, phosphorous, PTH levels are in normal limits. At the audiometric reassesment pure tone audiometry averages of air/bone conduc-tion are 22 dB/17 dB on the right side and 37 dB/32 dB on the left side. Her hearing loss has not changed significantly. The differenc-es between pure tone averages of two periods are minimal and can be audiometrist dependent.

Conclusion: Hearing loss due to Paget’s disease may not be re-versible but early diagnosis and treatment may prevent further deterioration of hearing. Randomized, double-blind, placebo con-trolled clinical trials in which the effect of antipagetic therapy is assessed on hearing loss in patients with Paget’s disease of the temporal bone, are needed.

Keywords: hearing loss, paget’s disease, bisphosphonate

O - 12 Bone/Calcium

effeCt Of zOlendranate treatment On Hearing lOss in a Patient WitH tHe Paget’s

disease WHO Has skull invOlvementiLKAy KARTAL1, ARzU TATLIPINAR2

1 Department of Internal meDIcIne, DIvIsIon of enDocrInology anD metabolIsm, meDenIyet UnIversIty, goztepe traInIng anD researcH HospItal, IstanbUl, tUrkey

2 Department of otolaryngology- HeaD anD neck sUrgery, fatIH sUltan meHmet traInIng anD researcH HospItal, IstanbUl, tUrkey

2016


Introduction: Primary hyperparathyroidism is a disorder caused by enlargement of one or more parathyroid glands. About 6-16% of parathyroid adenomas can be in an ectopic location, therefore, localization of parathyroid lesions can be challenging.

Case Report: A 74-year-old female patient was admitted to our hospital with weakness, fatigue and constipation. Her medical history included type 2 diabetes mellitus, hypertension and coro-nary heart disease. Laboratory studies revealed normal iPTH con-centration (56.9 pg/mL normal: 12-88 pg/mL) and normal serum biochemistry except elevated calcium level (13.15 mg/dL). Urinary calcium excretion was to be high (549.15mg/day). Based on clinical and laboratory findings, a final clinical diagnosis of primary hyper-parathyroidism was made.

Neck ultrasonography exam was planned and performed with the intention to localize the adenoma. A 14x10 mm, hyopoechoic solid lesion was located in the inferior pole of the right thyroid parenchy-ma. In addition to this nodule there were also several other sub-centimeter solid nodules scattered throughout both thyroid lobes. The hypoechoic solid nodules described in the inferior pole of the right thyroid lobe was biopsied with fine needle aspiration (fNA) under US guidance. The cytological exam revealed atypia of un-

determined significance. The following 99mTc-MIBI scintigraphy, SPECT–CT, neck MRI and 4-D neck CT did not depict any findings suggestive of a parathyroid adenoma. Under the light of these find-ings we considered that an intrathyroidal parathyroid adenoma was clinically possible and a second fNA biopsy with PTH washout was performed. PTH washout concentration was found to be conclusive for a parathyroid adenoma with the level of 2612 pg/mL.

Based on these findings surgical removal of the right thyroid lobe was planned. Intraoperative frozen exam revealed this nodule to represent a papillary thyroid carcinoma and a total thyroidectomy was performed after this result. Post-surgical definitive patholog-ical exam confirmed that this nodule was consistent with a para-thyroid adenoma instead of PTC. Post-operative serum PTH and calcium levels returned to normal values.

Discussion: Intrathyroidal parathyroid adenomas may cytologically mimic primary papillary thyroid carcinomas. In this case report, we aimed to underline the diagnostic value of PTH washout after fNA for suspicious intrathyroidal nodules.

Keywords: parathyroid adenoma, hypercalcemia, fine needle aspi-ration, parathyroid hormone

O – 13 Bone/Calcium

tHyrOid nOdule Or ParatHyrOid adenOma: a Case rePOrt

EDA çALIşKAN yILDIRIM, UğUR üNLüTüRK, NAfiyE HELVACI, MiyASE BAyRAKTARDIvIsIon of enDocrInology anD metabolIsm, Department of Internal meDIcIne, Hacettepe UnIversIty, ankara,

tUrkey

2016


Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by thromboses, resulting in thrombo-cytopenia. It consists of the pentad of microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fe-ver, and renal disease. It’s unclear what triggers TTP, but some factors may play a role such as: pregnancy, malignancies, infec-tions, surgery, blood and marrow stem cell transplant, chemother-apeutics, ticlopidine, clopidogrel, cyclosporine A, estrogens and quinine. The association between hyperthyroidism or antithyroid drugs with TTP is nonexistent in the literature; this case is the first in the literature to discuss possible hyperthyroidism or antithyroid medication triggered TTP.

Case: A fifty four years old female patient was diagnosed with toxic multinodular goiter and treated with propylthiouracil for two weeks and methimazole for a week before admission. Patient was admit-ted to emergency room with disseminated purpura, acute renal failure and lethargy. Her routine blood work revealed elevated cre-atinine [4.8 mg/dL (0.5-1.1 mg/dL)] and thrombocytopenia [13.000/mm3 (150.000-450.000/mm3)]. Coagulation tests were normal. Pe-ripheral blood smear revealed shistocytes; patient was diagnosed as TTP and was hospitalized by Hematology. TSH levels were low [<0.01 mIU/mL (0.35-5.5 mIU/mL)] and free T4 and T3 levels were

elevated [T4: 3.28 ng/dL (0.72-1.56 ng/dL), T3: 16.3 pg/mL (1.8-4.6 pg/mL)]. ADAMTS13 levels were low; ADAMTS13 inhibitor was high. During her treatment for TTP, she was consulted with En-docrinology for hyperthyroidism. She did not have any symptoms related to hyperthyroidism; hematology planned plasmapheresis twice daily and corticosteroid treatment for TTP. We did not plan any additional urgent treatment for hyperthyroidism, we decided to follow up the patient with plasmapheresis since antithyroid drug related TTP was a possibility. After the patient was in remission from TTP, plasmapheresis was stopped, we planned radioactive iodine treatment. She was discharged without any complaints. At 3 months follow-up, she was in remission for TTP and her thyroid function tests showed subclinical hyperthyroidism.

Discussion: Here we present a case of TTP possibly triggered ei-ther by hyperthyroidism or antithyroid drugs. Laboratory results show that TTP is acquired in this patient. It is not possible to dif-ferentiate between drug-induced or disease-induced TTP. Since drug-induced case is a possibility, we believe that it was relatively not safe to use antithyroid medication in this patient.

Keywords: toxic multinodular goiter, metimazole, thrombotic thrombocytopenic purpura

O - 14 thyroid

a Case Of tHrOmBOtiC tHrOmBOCytOPeniC PurPura in a tOxiC multinOdular gOiter

Patient On antitHyrOid mediCatiOnGÖKHAN TAzEGüL1, TAHiR SAyGIN ÖğüT1, HüMEyRA BOzOğLAN2, ÖzLEM DOğAN2,

NUSRET yILMAz2, OzAN SALiM3, RAMAzAN SARI2, HASAN ALi ALTUNBAş2, MUSTAfA KEMAL BALCI2

1 akDenIz UnIversIty, facUlty of meDIcIne, Department of Internal meDIcIne, antalya/tUrkey 2 akDenIz UnIversIty scHool of meDIcIne, Department of enDocrInology, antalya/tUrkey

3 akDenIz UnIversIty scHool of meDIcIne, Department of Hematology, antalya/tUrkey

2016


Introduction: Papillary microcarcinomas can rarely manifest as aggressive bone metastatic lesions with unexpected laboratory and clinical findings. We herein present an elderly patient diagnosed with multiple bone metastasis during investigations for acute kid-ney injury.

Clinical Case: In february 2015, a 72-year-old woman experienced a car accident, which lead to right humerus fracture and acute kid-ney injury. Her previous history was remarkable for tuberculosis, hydatid cyst, type 2 diabetes and hypertension, and she donated her kidney to her daughter. She also suffered from widespread bone pain for 1 year, and abdominal CT revealed multiple bone lesions involving lumbar spine and ischial bone. Pelvic MRI and bone scan showed extensive lytic bone lesions involving mandible, humerus, femur, multiple ribs, manubrium sterni, scapula, clav-icle, lumbar and sacral spine and pelvic bones. Bone biopsy was compatible with thyroid cancer. Thyroid ultrasound revealed mul-tiple nodules, and biopsy was suspicious for follicular neoplasm. The patient underwent total thyroidectomy and neck dissection, and histopathological investigations showed two foci of follicular variant of papillary microcarcinoma in the right lobe. External ra-diotheraphy was also performed for some of the metastatic bone lesions. Postoperatively, she was treated with 200 mCi 131I-ther-apy combined with temporary high dose dexamethasone. Whole body scan after treatment showed uptakes in the neck and bones. Levothyroxine was begun and the dose was increased to 150 mcg/day, but the patient began to experience dyspnea and pretibial ede-

ma due to diastolic congestive heart failure. Thyroid function tests showed a very low fT4, high fT3 and supressed serum TSH. During follow-up L-thyroxine dose was tapered to 50 mcg/day. Her weird thyroid function tests were possibly due to tumor tissue expressing excessive type 2 deiodinase activity converting circulating T4 to T3. She did not have functioning thyroid cancer metastases, since the patient’s thyroid hormone levels fell when levothyroxine supple-mentation was held, and her TSH levels were increased. After the first radioactive iodine therapy she experienced progressive tumor burden, and another 200 mCi radioactive iodine was administered, which resulted in tumor lysis syndrome, DIC, bone marrow failure, and she needed hemodialysis. She rejected further treatment and died of extensive metastatic disease at home.

Conclusion: Although papillary microcarcinomas are known to be tumors with good prognosis, they can also present with extensive refractory and fatal bone metastasis. Some of the weird thyroid function tests such as low free T4 and normal to high free T3 levels and comparably low levothyroxine need to supress TSH may be as-sociated with type 2 deiodinase activity in the tumor. In these condi-tions, increasing levothyroxine dosage for normalising free T4 level is useless and may even lead to very high T3 levels and associated adverse affects.

Keywords: follicular variant of papillary microcarcinoma, bone metastasis, thyroid function tests

O - 15 thyroid

agressive fOlliCular variant Of PaPillary miCrOCarCinOma Presenting WitH extensive BOne metastases and assOCiated WitH Weird

tHyrOid funCtiOn testsNURDAN GüL1, HüLyA HACIşAHiNOğULLARI1, METBAN MESTANzADE1, AyşE KUBAT üzüM1,

ÖzLEM SOyLUK SELçUKBiRiCiK1, SEVGi KALAyOğLU BEşIşIK2, REfiK TANAKOL1

1 IstanbUl facUlty of meDIcIne, Department of Internal meDIcIne, DIvIsIon of enDocrInology anD metabolIsm , IstanbUl, tUrkey

2 IstanbUl facUlty of meDIcIne, Department of Internal meDIcIne, DIvIsIon of Hematology , IstanbUl, tUrkey

2016


Introduction: Thyrotoxic periodic paralysis (TPP) is characterized by sudden onset of hypokalemia and paralysis under the condition of thyrotoxicosis. TPP is more prevalent in Asians than in non-Asians and also in males more than in females. In Chinese and Japanese patients with hyperthyroidism, 1.8–1.9% experience TPP. The typical age of onset is 20–40. Hypokalemia in TPP results from an intracellular shift of potassium under conditions of high thyroid hormone levels.

Clinical Case: A 29-year-old man with a history of recurrent mus-cle weakness presented to our emergency department with diffi-culty in walking. The patient’s symptoms started one day before, and he was unable to walk to the hospital. He had had similar epi-sodes before. He stated palpitations, tremor and excessive sweat-ing. He had no family history of periodic paralysis.

On physical examination, he had an enlarged thyroid gland and tachycardia. He had tremor in his hands and decreased mus-cle strength in both lower extremities. His initial potassium lev-el was 2.6 mEq/L (3.5–5.1 mEq/L), phosphorus level was 3.7 mg/dL (2.5–4.5 mg/dL), and magnesium level was 1.9 mg/dL (1.7–2.6 mg/dL). Electrocardiogram showed sinus tachycardia with a rate of 124 beats per minute. He was given intravenous potassium chloride with saline infusion. He was also given oral potassium replacement, which resulted in resolution of his lower-extremity paralysis. On the second day of admission, his serum potassium level increased to 4.8 mEq/L. His renin and aldosterone levels were

within normal limits. The thyroid-stimulating hormone (TSH) level was 0.01 mU/L (0.4–3.9 µIU/mL), the free thyroxine (fT4) level was 3.8 ng/dL (0.8–2.7 ng/dL) and the free triiodothyronine (fT3) level was 13.8 ng/dL (2.3–4.4 pg/mL). Thyrotoxicosis was diagnosed, and the patient was given propranolol 40 mg twice a day. The anti-TSH receptor antibody level was 19.6 IU/L (1.22-1.75 IU/L), His thyroid scintigraphy and uptake scan was consistent with Graves’ disease. He was started on methimazole 20 mg per day. Serial measure-ments of his serum potassium level remained within normal limits without potassium replacement. The patient was diagnosed as TPP secondary to Graves’ disease.

CONCLUSION: The diagnosis of TPP is made when a patient pres-ents with a paralytic attack that is associated with hypokalemia and hyperthyroidism. The most common underlying form of thyroid dis-ease associated with TPP is Graves’ disease but any other cause of hyperthyroidism can be associated with TPP. The condition may be life-threatening if weakness of the breathing muscles leads to respiratory failure, or if the low potassium levels lead to cardiac arrhythmias. Patients should be on cardiac monitoring, and potas-sium levels should be monitored for potential rebound hyperkale-mia. Treatment of TPP includes replacing potassium, prevention of this shift of potassium by using nonselective beta-blockade and treatment of underlying thyrotoxicosis.

Keywords: thyrotoxicosis, hypokalemia, paralysis

O - 16 thyroid

tHyrOtOxiC PeriOdiC ParalysisETHEM TURGAy CERiT1, MEHMET TUNCAy2, MEHMET MUHiTTiN yALçIN1

1 Department of enDocrInology & metabolIsm, Dr. ersIn arslan traInIng anD researcH HospItal, gazIantep, tUrkey

2 Department of nepHrology, Dr. ersIn arslan traInIng anD researcH HospItal, gazIantep, tUrkey

2016


Introduction: Hypothyroidism and pleural effusion (PE) are well-defined to accompany, however thyrotoxicosis and PE collab-oration is not so frequent. We described a patient with no history of heart or liver failure; suffering from PE, peripheral edema, and hypoalbuminemia who was diagnosed with thyroxicosis. PE and peripheral edema resolved immediately after initiation of antithy-roid medication.

Case- A 50-year old man arrived at our clinic with fatique, short-ness of breath, palpitations and edema in lower extremity. He had a pulse rate of 130/min with a respiratory rate of 28/min, while pulmonary examination defined decreased sounds in right inferi-or and mid lobe and there was grade 3 pretibial edema. His blood test results were: Creatinine: 0,32 mg/dl, Albumin(alb): 2.8 g/dl, Alanine Aminotransferase (ALT): 21 U/l, Gamma Glutamyl Trans-ferase (GGT): 48 U/l, Thyroid Stimulating Hormone (TSH): 0.00 µIU/ml, free T4 (fT4): 3. 6 (0.89-1.79) ng/dl, free T3 (fT3): 8.7 (2.3-4.2) pg/ml. His chest graph revealed right pleural effusion and chest computed tomography (CT) showed 27 mm pleural effusion in right hemithorax. Echocardiography (echo) demonstrated grade 2 mitral regurgitation (MR), tricuspid regurgitation (TR) and normal ejection fraction. Hepatobiliary ultrasonography (USG) showed no pathology. Spironolactone 25 mg/d, hydrochlorthiazide 25 mg/d and furosemide 40 mg/d was started. Thyroid USG revealed mul-tinodular goiter, the biggest nodule 18x22x34mm on right lobe and thyroid 99m Technetium-pertecnatate scintigraphy showed diffuse

increased uptake and a hypoactive nodule in right lobe. After toxic diffuse multinodular goitre (TDMNG) was diagnosed, MMI 30 mg/day was attempted to start. Since unavailability of MMI, PTU 300 mg/day was started. After one week, fT4: 2.55 ng/dl, fT3: 8.37 pg/ml were found along with increase of alb to 3.5 g/dl. Meanwhile pulmonary sounds reversed to normal and chest graph revealed normal examination with peripheral edema resolved in lower ex-tremity. Echo showed trace amount of TR and MR. After one month TSH:0.00 µIU/ml, fT4: 2.24 ng/dl, fT3: 7.95 pg/ml and 3.8 g/dl alb level were seen. A month later, alb level rised to 4.6 g/dl.

Conclusion: PE is rare, while hypoalbuminemia is again unex-plained in thyrotoxic patients. Thyrotoxic state establishes inflam-mation that would cause alb to decrease. In our case hypoalbu-minemia evolved to be noteworthy since it would describe PE and peripheral edema accompanying thyrotoxicosis. After antithyroid treatment, volume excess resolved with alb rise. Nevertheless, multiple diuretic medications may also improve the patients’ ail-ments. Diuretics may have decreased the volume overload and me-diate alb level rise by increasing alb concentration. However abrupt disappearance of such volume load would not be depended only on low dose oral diuretic treatment. PE may be seen in patients with thyrotoxicosis and responds well to antithyroid therapy and low dose diuretic therapy.

Keywords: Thyrotoxicosis, pleural effusion, hypoalbuminemia

O - 17 thyroid

tHyrOtOxiCOsis WitH Pleural effusiOn and HyPOalBuminemia

iffET DAğDELEN DURAN1, NEşE ERSÖz GüLçELiK2

1 Department of enDocrInology anD metabolIc DIseases, kIrIkkale yüksek İHtIsas HospItal, kIrIkkale, tUrkey 2 Department of enDocrInology anD metabolIc DIseases, ankara eDUcatIon anD researcH HospItal,

ankara,tUrkey

2016


Introduction: Graves’ disease is an autoimmune disorder which can be accompanied with other autoimmune diseases affecting various organ systems. In such cases, treatment of hyperthyroid-ism may be troublesome due to organ dysfunction caused by con-comitant disease. Herein, we present a patient with liver failure due to primary sclerosing cholangitis complicated with Graves’ disease.

Case Presentation: A 35-year-old man, who was diagnosed with friedreich Ataxia at the age of 12 and primary sclerosing cholangi-tis two months ago, presented to our gastroenterology clinic with the complaints of pruritus, diarrhea, palpitations and weight loss. He was currently taking ursodeoxycholic acid and proton pump inhibitor ther-apy. On physical examination, he had jaundice, ataxic gait, choreiform movements. Laboratory workup yielded elevated ammonia levels indicating hepatic encephalopathy in addition to very high bilirubin, transaminase and prothrombin time levels. Thyroid function tests suggested primary hyperthyroidism. TSH receptor antibody (TRAb) was positive (6.27 IU/L). Thyroid ultrasonography showed parenchy-mal heterogeneity in a normal sized gland, which was diffusely hyper-

active in technetium 99m pertechnetate scintigraphy. The patient was diagnosed as Graves’ disease. Antithyroid druq therapy was not suit-able for the patient because of liver failure. Therefore, the patient un-derwent plasmapheresis as a temporary management option before definitive treatment for thyrotoxicosis. After two sessions of plasma-pheresis, TRAb became negative, fT4 and fT3 levels decreased, clinical findings of hepatic encephalopathy improved due to decreasing biliru-bin levels and the need for urgent liver transplantation disappeared. After discharge, total thyroidectomy was performed. Pathological ex-amination revealed diffuse hyperplasia and multifocal thyroid micro-papillary carcinoma.

Conclusion: Plasmapheresis is a viable option for patients with Graves’ disease and impaired liver function. In our case, only two sessions of plasmapheresis enabled the patient to have thyroid surgery and resolved the need for urgent liver transplantation.

Keywords: Plasmapheresis, Graves’ disease, primary sclerosing cholangitis

O - 18 thyroid

PlasmaPHeresis as a temPOrary management OPtiOn in a Patient WitH graves’ disease and

Primary sClerOsing CHOlangitisBATUHAN BAşPINAR1, SEDA HANifE OğUz2, TAyLAN KAV3, BüLENT OKAN yILDIz2

1 Department of Internal meDIcIne, Hacettepe UnIversIty scHool of meDIcIne, ankara, tUrkey 2 DIvIsIon of enDocrInology anD metabolIsm, Department of Internal meDIcIne, Hacettepe UnIversIty scHool of

meDIcIne, ankara, tUrkey 3 DIvIsIon of gastroenterology, Department of Internal meDIcIne, Hacettepe UnIversIty scHool of meDIcIne,

ankara, tUrkey

2016


Introduction: Papillary thyroid carcinoma (PTC) is the most com-mon malignancy of the thyroid gland. Metastasis to the regional lymph nodes is relatively high, and the most common distant me-tastasis sites are the lung and bone. Skin metastasis of PTC is very rare, whereas scalp is the most frequent cutaneous area involved.

Case Presentation: 80-year-old female with history of hyperten-sion and diabetes was referred to neurosurgery clinic due to a pro-gressively enlarging lump at the frontal head region for 4 years. CT scan revealed a heterogeneously enhancing semisolid lesion measuring 115x84x69 mm. The lesion was found to be causing ex-tensive frontal bone destruction with associated superior sagittal sinus invasion. Incisional biopsy of the lesion came back as well- differentiated thyroid carcinoma metastasis with positive staining for TTf-1 and thyroglobulin. Her TSH level was suppressed with a value of 0.18 mIU/ml and with normal fT4 and fT3 levels (10.56 pmol/L, fT3: 5.94 pmol/L, respectively). Thyroid ultrasonography exam revealed a left lobe located hypoechoic solid nodule with pe-ripheral coarse eggshell calcifications measuring 30x20 mm. This nodule was found to be cold on thyroid scintigraphy. US guided fine needle aspiration biopsy of the nodule was reported to be highly

suspicious for follicular thyroid carcinoma. Genomic studies re-vealed negative Pax8-PPARg gene rearrangement. PET-CT study performed after the biopsy did not detect any additional metastatic foci. The patient underwent total thyroidectomy and final patholog-ic examination revealed a 4 cm diameter and encapsulated type papillary thyroid carcinoma of follicular variant. The serum fT4 and fT3 levels remained elevated after total thyroidectomy (with the lev-els of 37.78 pmol/L and 12.04 pmol/L, respectively) a finding which we ascribed to continued, and uncontrolled, thyroid hormone pro-duction by the metastatic head lesion.

Based on these clinical and laboratory findings, we started oral methimazole therapy. After consultation with neurosurgery de-partment the lesion was planned for cytoreductive surgery as the patient was not a candidate for complete resection.

Discussion: Cutaneous thyroid carcinoma metastasis is highly unusual and it generally occurs in the setting of disseminated ad-vanced stage disease.

Keywords: papillary thyroid carcinoma, skin metastasis,scalp

O – 19 thyroid

a lumP On tHe sCalP: an unusual PresentatiOn Of PaPillary tHyrOid CarCinOma

DAMLA EyüPOğLU1, UğUR üNLüTüRK2

1 Department of Internal meDIcIne, Hacettepe UnIversIty, ankara, tUrkey 2 Department of enDocrInology anD metabolIsm, Hacettepe UnIversIty, ankara, tUrkey

2016


Introduction: Wernicke encephalopathy (WE) is an acute neurologic disorder and results from deficiency of thiamine (vitamin B1). Tradi-tional clinical triad is occulomotor abnormalities, confusion and gait ataxia. It has a high chance of recovery through thiamine treatment. However, since there is a serious mortality risk, conditions other than alcoholism which may lead to malabsorption should be included in the differential diagnosis. In this report, a WE case which developed after Roux-en-y gastric bypass surgery due to morbid obesity is presented.

Case Report: A female patient, aged 47 and known to have had dia-betes mellitus for 17 years and getting insulin treatment, underwent a gastric bypass operation in November 2014 since BMI was 51.4 kg/m2. After two months from the operation, the patient was hospitalized due to dizziness, fatique, nausea and vomiting. After 6 months from the operation, the patient was observed to have lost 53 kg.

In the physical examination, the general condition of the patient was good; and there were pearlescent striae and surgical scars on the abdomen. In the neurological examination, the patient was con-scious, cooperative, oriented and had end-point nystagmus. Deep tendon reflexes were symmetrical and normal, romberg test was negative but ataxia was present.

In biochemical examination, Hba1c was %7.6, Hb was 9.7 g/dl, cre-atinine was 1.12 mg/dL, and iron parameters, B12 and folic acid were normal. In cranial MRI, multiplane monitoring was carried out at TSE (turbo spin echo) and fLAIR (fluid-attenuated inversion recovery) sequences. In supratentorial sections, on T2-weighted and fLAIR images hyperintensity was detected in mamillary body and periaqueaductal area. In diffusion weighted MRI, acute isch-emia or diffusional restriction were not detected. With reference to the MRI findings, WE was thought to be present in the patient who had rapid weight loss after gastric bypass surgery. Intravenous thiamine treatment 500 mg/day was started. It was given for two days and then the treatment continued with oral administration of vitamine B. The symptoms were regressed significantly in a week.

Conclusion: Roux-en-y gastric bypass is a bariatric surgery which gives good results in the long term. However, in the post-operative pe-riod nausea and vomiting occur frequently. Wernicke Encephalopathy

is a disorder resulting from a combination of ataxia, ophthalmople-gia, nystagmus, mental confusion and lack of vitamin B1. Thiamine deficiency can be seen in alcoholism, prolonged fasting, hyperemesis gravidarum, prolonged parenteral nutrition, dialysis and AIDS. If Wer-nicke Encephalopathy is not treated, it can cause catastrophic amne-sia, Korsakoff’s psychosis and death. In conclusion, like in this case, it should be kept in mind that Wernicke Encephalopathy may be seen in patients who have complaints of dizziness after gastric bypass.

Keywords: Wernicke encephalopaty, thiamine, gastric bypass

figure 1. Periaquaductal involvement in axial T2

figure 2

figure 2. Mamillary body in T2 fLAIR(marked with pink arrow) and periaquaductal hyperintense areas(marked with yellow arrow)

O – 20 lipids/Obesity

WerniCke enCePHalOPatHy in a Case WHO Had gastriC ByPass surgery due tO mOrBid OBesityHATiCE ÖzIşIK1, BANU şARER yüREKLi1, NiLüfER ÖzDEMiR KUTBAy1, ILGIN yILDIRIM şiMşiR1,

CAN EMRE ERDOğAN2, füSUN SAyGILI1

1 Department of enDocrInology, ege UnIversIty, İzmIr, tUrkey 2 Department of general neUrology, ege UnIversIty, İzmIr, tUrkey

2016


Background: Celiac disease (CD) is a malabsorption syndrome. Its coexistence with type 1 diabetes mellitus (t1DM) has been proven. Growth retardation is the most common cause that leads to the diagnosis in childhood and adolescence.

Clinical Case: A 7.5-year-old girl presented with hematuria and poly-uria. She was born at term after an uneventful pregnancy with normal vaginal delivery (birth weight: 3720gr). Her height followed the 97th percentile (her target height was at 75th percentile). Since then, her weight increased inappropriately due to unhealthy diet and exercise absence. At the admission her BMI was 24.4 (>97th centile). She devel-oped body odor and pubic hair at the age of 7 years. Both her parents had constitutional delay of growth and puberty. Regarding her family history, her mother and maternal grandmother had nephrolithiasis, her maternal grandfather had t2DM and her father had hypertension. Her mother also reported a history of gestational diabetes during her second pregnancy. On physical examination, she was prepubertal (Tanner stage: Breast 1, Pubic Hair 2, Axillary Hair 1) and acanthosis nigricans was noticed. Her initial investigation tests revealed cystitis and severe hypeglycemia. Her HbA1c level was 9.1% and she was put on insulin treatment and antibiotics. Due to obesity and family history of diabetes, it was ordered further laboratory workup that supported

the diagnosis of t1DM by revealing very high Anti-GAD and Anti-insu-lin antibodies levels. Moreover, she was checked for Hashimoto thy-roiditis and CD and was found very high Antigliadin IgG, Anti-tTG IgA and Anti-endomysial antibodies levels. HLA class II typing revealed the presence of DQA1*05 and DQB1*02 alleles that encode HLA-DQ2 heterodimer found in approximately 95% of patients with CD. Subse-quently, gastroscopy led to the diagnosis of CD. Her bone age was 2.5 years ahead of chronological age. She was prescribed a specific diet and exercise program that led to weight loss while two months later, following the CD diagnosis, she was put on gluten free diet. As soon as she left hospital, her insulin requirements decreased and 20 days after the onset of therapy she stopped insulin due to repeated hypogly-cemic episodes. Her HbA1c level was within normal range 4 months later and she was off insulin treatment for 8 months. After that period hyperglycemic episodes were noticed, HbA1c level increased and she was put on insulin treatment again.

Conclusion: Even though obesity is very uncommon between pa-tients with CD, it is necessary for every patient with t1DM to be evaluated for CD at the time of t1DM diagnosis and on regular basis thereafter.

Keywords: diabetes, celiac disease, obesity

O - 21 diabetes

tHe investigatiOn fOr CeliaC disease in a neWly diagnOsed Patient WitH diaBetes is

neCessary in any CaseMARIA PAPAGIANNI1, GEORGE PALTOGLOU2, GEORGE MASTORAKOS3

1 enDocrIne UnIt, tHIrD Department of peDIatrIcs, arIstotle UnIversIty of tHessalonIkI, HIppokrateIon general HospItal of tHessalonIkI, tHessalonIkI, greece

2 fIrst Department of peDIatrIcs, “agHIa sofIa” cHIlDren’s HospItal, atHens UnIversIty meDIcal scHool, atHens, greece

3 enDocrIne UnIt, seconD Department of obstetrIcs anD gynecology, areteIon HospItal, atHens UnIversIty meDIcal scHool, atHens, greece

2016


Mauriac syndrome (MS) is an underrecognized complication, char-acterized by short stature, delayed puberty mostly in adolescents with poor control of type 1 diabetes metllitus (T1DM). It presents with cushingoid features and hepatomegaly due to hepatic glucog-enosis with altered liver enzymes.

A 20-year-old female with a-17 year history of T1DM was admit-ted with diabetic ketoacidosis and for further evaluation of her lat-est poor glycemic control and abdominal distension, she had for a long time. The patient was on intensive insulin treatment since her disease onset. Her first diabetic ketoacidosis was documented in 2014. Her present admission was due to her third diabetic ketoac-idosis. Her ever lowest HbA1c was 8%, but last several years her A1c levels have been measured between 9.2-11%.

Anthropometric data revealed height 146 cm (<3 rd percentile), weight 44 kg (<3 rd percentile), body mass index 20.6 kg/m2. The patient menarch age was 18 and she was pubertal with Tanner stage III for breast and pubic hair development.

She complained about the distention and tenderness due to her pro-tuberant abdomen, and physical examination revealed tender and 4-5cm enlarged liver below her rib cage. Additionally, she had Cush-ingoid features; moon face, high pitched voice and abdominal obesity.

Dynamic liver MRI, due to her distention and tender liver, demon-strated enlarged right lobe of liver of 17cm size, left lobe reaching and covering across her spleen.

Lab Results: ALT: 101 (0-35), AST: 83( 0-35), A1c:9.4%, fasting plasma glucose:374. Liver biopsy revealed swollen hepatocytes and glycogenic hepatopathy, which ensured, a rare complication of T1DM, the Mauriac syndrome.

Evaluation for microvascular complications demonstrated no dia-betic retinopathy and nephropathy. Besides, no diabetic neuropathy was detected. The average blood pressure was 110/65mmHg.

After glycemic control was established, her distention and liver tenderness were regressed. As a result of phycological assesment treatment due to anxiety was initiated. Liver enzymes before dis-

charge were: ALT:75, AST:61. A1c:8.2%.

MS is a syndrome of growth failure and delayed puberty associated with massive liver enlargement from glycogen deposition in pa-tients with poorly controlled T1DM, which is rarely seen nowadays. Liver biopsy should confirm the hepatic glucogen deposition.

A mechanistic cause for this syndrome is unknown. However, this year a mutation in the catalytic subunit of liver glycogen phosphory-lase kinase was discovered by MacDonald et al. The effect of a mutant enzyme of glycogen metabolism can combine with hyperglycemia to directly hyperinhibit glycogen phosphorylase, in turn blocking glycog-enolysis causing the massive liver in Mauriac disease.

The main treatment of Mauriac syndrome is the amelioration of glycemic control that is usually accompanied by the reversal of the liver disorder.

The result of genetic test of this patient is pending.

Keywords: type 1 diabetes mellitus, mauriac syndrome, hepatic glycogen deposition

figure 1: Dynamic Liver MRI

O – 22 diabetes

mauriaC syndrOme still exists; a rare COmPliCatiOn Of tyPe 1 diaBetes mellitus

ANARA KARACA, ELif TOPçUOğLU, NEşE ERSÖz GüLçELiK, TüLAy OMMA, NUJEN çOLAK BOzKURT, SEVDE NUR fIRAT, TAHSiN ÖzENMiş, GÖNüL KOç, CAViT CULHA, yALçIN ARAL

Department of enDocrInology anD metabolIsm, ankara teacHIng anD researcH HospItal, ankara, tUrkey

2016


Primary bilateral macronodular adrenal hyperplasia (BMAH) is a rare condition, characterized by enlarged adrenal glands and se-cretion of cortisol independent of pituitary adrenocorticotropic hormone (ACTH) which results in subclinical or overt Cushing syn-drome (CS). Bilateral adrenalectomy is the standard of care in overt CS but it imposes primary adrenal insufficieny and requires life long glucocorticoid replacement. We describe a patient with BMAH who underwent unilateral adrenalectomy of the enlarged adrenal gland and subsequently displayed remission of CS.

57 year old woman with a history of type 2 diabetes mellitus (DM) and hypertension for 5 years, atrial fibrillation and ischemic stroke for 3 years admitted to our clinic because of bilateral adrenal en-largement on MRI that’s seen due to abdominal pain. On physical examination, patient was found to have centripedal obesity with BMI 34 kg/m², plethora and buffalo hump. Her blood pressure was 140/80 mmHg under ramipril 5 mg and carvedilol 12,5 mg treat-ment. With metformin 2000 mg, insulin aspart 3x10 ü and insu-lin detemir 1x16 ü the patient’s HbA1C level was %6,7. Contrast enhanced CT revealed multinodular enlargement of both adrenal glands with left side predominance and the largest nodules were 40x40 mm on left side and 22x20 mm on right side.

Endocrine assesment showed 3-4 fold increased urinary cortisol lev-els, loss of diurnal rhythm with elevated late night serum and salivary cortisol levels. Plasma cortisol was not suppressed in a 2 mg dexa-metasone suppression test (DST). Plasma ACTH level was < 5 pg/

ml showing the etiology of CS as adrenal gland. 24 hour urinary ca-thecholamines, 17-OH progesterone and DHEA-S levels were normal. Tests for aberrant receptor expression was normal. With the diagnosis of CS due to BMAH we decided on unilateral adrenalectomy of the pre-dominantly enlarged gland and the patient underwent laparoscopic left adrenalectomy. On macroscopic examination adrenal gland size was 9,5x5x4,5 cm consisting of multipl yellow nodules. Microscopic examination revealed multiple nodules composed of lipid rich clear cells and Ki-67 proliferation index was %2.

Serum cortisol was 4,3 µg/dl on post operative day 1 and hydrocor-tisone 25 mg was started. Two months after surgery ACTH stimula-tion test was normal and hydrocortisone was stopped. One month later urinary cortisol, late night salivary cortisol were normal and cortisol was suppressed in response to 2 mg DST and CS was in remission. At the last visit 8 months after surgery the patient was in remission, lost 12 kg weight and DM was treated with metformin alone without insülin requirement and HbA1C level was %5.8. We follow-up patient with six months intervals.

Unilateral adrenalectomy of the larger gland appears to be an ef-fective treatment for BMAH. It may result in remission of CS and improves obesity and diabetes. But follow-up of the patient is re-quired for recurrence of the disease.

Keywords: Cushing syndrome, primary bilateral macronodular adrenal hyperplasia, unilateral adrenalectomy

O – 23 adrenal

unilateral adrenaleCtOmy imPrOves CusHing syndrOme in a Patient WitH Primary Bilateral

maCrOnOdular adrenal HyPerPlasiaALPER çAğRI KARCI, zEyNEP çETiN, NARiN NASIROğLU iMGA, DiLEK BERKER, SERDAR GüLERDepartment of enDocrInology anD metabolIsm, ankara nUmUne eDUcatIonal anD researcH HospItal, ankara,

tUrkey

Table 1. Laboratuary tests for Cushing syndrome evaluation

AT DIAGNOSIS POST-OP 3 MONTHS POST-OP 8 MONTHS

ACTH (pg/ml) <5 // <5 30 24

Cortisol (mcg/dl) 17,4 16,2 15,8

24-hour urinarycortisol (3,5-45 mcg/day) 156 // 210 40 34

Late-night salivarycortisol(0,2-2,2 ng/ml) 6,8 // 8,83 2,2 1,89

2 day-2mg DST 10,8 1,83 1,68

2016


Background: Congenital adrenal hyperplasia (CAH) is one of auto-somal recessive disorders resulting from CyP 21(21-hydroxylase) deficiency. Testicular adrenal rest tumors (TARTs) are common in CAH, due to hyper-secretion of ACTH. These lesions inside the tes-tis are bilateral and multiple. TARTs may lead to structural damage and their tumors may be mistaken for Leydig cell tumor. Because of their locations; TART may cause seminiferous obstruction and infertility.

History: 6-year old boy presented with early growth of pubic hair, the condition started at age of four years when his parents noticed that their child has developed pubic hair. They also noticed that he is taller than his siblings with abnormal behavior and deepening of voice. There is history of sudden death of his brother when he was 22 days old. Testicular examination was normal. Physical ex-amination revealed develpoment of pubic hair (stage 4) and adult scrotum.

Diagnosis and Treatment: His random 17OH progesterone was 60.80 mg/ml (increased by more than 60 folds, repeated result for 17OH progesterone showed increase by more than 20 folds). U/S and MRI showed testicular tumors keeping with TARTs. We started patient on HC tabs with consultation of urologist . Continuous fol-low up of these tumors were done.

Conclusion: Giving the clinical presentation, biochemical profile and MRI findings, the diagnosis is keeping with CAH complicated with TARTs. Multidisciplinary approach with the involvement of en-docrinologist, pathologist and urologist is of great importance for the correct diagnosis and treatment. The aim is to draw the atten-tion of the clinicians to the presence of the TARTs and to evaluate critically every patient with testicular tumors concomitantly with CAH. It’s very important to consider treatment of CAH to prevent short stature in future.

Keywords: CAH, TART, precocious pubery

O – 24 adrenal

CaH Presenting as Premature PuBerty WitH assOCiated testiCular adrenal rest tumOrs

(tart)BAyAR AHMED QASIM

Dpartment of meDIcIne, UnIversIty of DUHok, Iraq

2016


Introduction: Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. About 42% to 57% of patients with ACC pres-ent with symptoms of hormonal excess such as Cushing syndrome or virilization. Non-functional tumors usually present with abdom-inal or flank pain, varicocele and renal vein thrombosis. In addition, a growing proportion of these patients (>15%) is initially diagnosed incidentally. Here we report 3 ACC cases with different clinical pre-sentations.

Case 1: A 38-year-old female patient admitted to our clinic with low back and abdominal pain, weight loss, and hirsutism. On phys-ical examination, her body mass index was 17.3 kg/m2 and ferri-man-Gallwey score (fGS) was 10. She had a history of vena cava inferior (VCI) thrombosis and a filter was put in another center 6 months ago. Abdominal ultrasonography (US) revealed invasion and thrombosis in VCI and multiple metastatic lesions with the big-gest diameter of 58 mm in the liver. In computerized tomography (CT), a 200x95 mm lesion in the left adrenal gland was observed. Basal cortisol and ACTH levels were 11.4 mcg/dL and 4.6 mcg/dL, respectively. Overnight 1 mg and 2 days 2 mg dexamethasone sup-pression tests (DSTs) showed no suppression in the morning fast-ing cortisols. DHEAS was 907 mcg/dl (normal limits: 0-340 mcg/dl) (Table 1). The percutaneous biopsy of metastatic mass in the liver revealed ACC and the patient was evaluated as inoperable.

Case 2: A 57-year-old female patient admitted to internal medicine clinic with fatigue, abdominal pain and high blood pressure. She was referred to our clinic due to low potassium (3.1mEq/L) concen-tration. In further laboratory examination, basal cortisol was 11.8 mcg/dL, ACTH was 2.6 mcg/dL, DHEAS was very high (1000 mcg/dl) and plasma aldosteron/renin ratio was normal. Abdominal CT revealed a 110x88 mm mass in the right adrenal gland. There was no suppression in overnight 1 mg and 2 days 2 mg DSTs (Table 1). Patient underwent right adrenalectomy and pathology was consis-tent with ACC.

Case 3: A 52-year-old female patient admitted to our clinic with hirsutism in the last 6 months. On physical examination, her body mass index was 31 kg/m2 and plethora, moon face, abdominal obe-sity, abdominal blue-purple striae, and buffalo hump were present. Basal cortisol and ACTH were 23.0 mcg/dl and 1.0 pg/ml, respec-tively. DHEAS was 884 mcg/dl. Overnight single dose of 1 mg and 2 days 2 mg DSTs showed no suppression (Table 1). Adrenal Cush-ing’s syndrome was diagnosed. A 80x60 mm mass in the left adre-nal gland was observed in abdominal CT. Right adrenalectomy was performed and the histopathological diagnosis was ACC.

Conclusion: ACC is seen more commonly, diagnosed at a young-er age and more often functional in females compared to males. The clinical presentation varies in a wide spectrum ranging from asymptomatic/mild disease as in case 3 to symptoms related with hormone overproduction as in case 2 and to severe life threating metastasis as in case 1.

Keywords: adrenocortical cancer, cushing syndrome, inferior vena cava trombosis, hypokalemia

Figure 1

Adrenal masses in patients Case 1: 200x95 mm heterogeneous ne-crotic hypodense solid lesion in the left adrenal gland (CT) Case 2: 110x88 mm heterogeneous necrotic solid lesion in the right adre-nal gland (CT) Case 3: 80x60 mm heterogeneous solid lesion in the left adrenal gland (CT)

O – 25 adrenal

different CliniCal PresentatiOns in tHree Patients WitH adrenOCOrtiCal CanCerNAGiHAN BEşTEPE1, DiDEM ÖzDEMiR1, şEfiKA BURçAK POLAT1, MEHMET KILIç2,

AyDAN KILIçARSLAN3, MELiKE RUşEN METiN4, REyHAN ERSOy1, BEKiR çAKIR1

1 yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, Department of enDocrInology anD metabolIsm ankara, tUrkey 2 yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, Department of general sUrgery, ankara, tUrkey

3 yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, Department of patHology, ankara, tUrkey 4 atatürk eDUcatIon anD researcH HospItal, Department of raDIology, ankara, tUrkey

2016


Table 1: Clinical, laboratory and radiological findings of patients

Case 1 Case 2 Case 3

Age /sex 38/female 57/female 52/female

Presentation Abdominal pain, weight loss, hirsutism, inferior vena cava thrombosis

fatigue, abdominal pain high blood pressure, hypokalemia

Hirsutism, moon face, blue-purple striae, buffalo hump

Basal cortisol (mcg/dl) 11.4 11.8 23

ACTH (pg/ml) 4.6 2.6 1

Overnight DST (mcg/dl) 10.2 12.4 24

Low dose DST (Liddle) (mcg/dl) 13.1 12.9 25.8

8 mg DST (mcg/dl) 12.6 13.2 23.2

24 hour urinary catecholamines Normal Normal Normal

Aldosterone/renin ratio Normal Normal Normal

DHEAS04 (0-340mcg/dl) 907 1000 884

17-OH progesterone (0.27-1.99) (ng/ml) 6.2 2.5 0.8

Adrenal imaging 200x95 mm heterogeneous necrotic hypodense solid lesion in the left adrenal gland, metastatic lesions (max 58 mm) in the liver

110x88 mm heterogeneous necrotic solid lesion in the right adrenal gland

80x60 mm heterogeneous solid lesion in the left adrenal gland

2016


Case Presentation: A 60 years old Caucasian heavy smoker man presented with cough and backache for four months as well as gradually worsening weakness, intermittent abdominal pain and slight hyperpigmentation. Because of the above, the primary care physician from a Greek island where the patient lived ordered a CT, which incidentally revealed bilateral lesions in adrenal glands (8 cm R and 7 cm L) (fig. 1). He was then referred to our hospital for further investigation and treatment. On admission, the patient presented with dehydration, hyperpigmentation, cachexia and or-thostatic hypotension. Laboratory evaluation revealed: Glu=115mg/dl, Creat=1.9mg/dl, K=5.7mEq/l, Na=121mEq/l, f=5 μg/dl, ACTH=831pg/ml, DHEAS=13mg/dl and Renin=217 pg/ml. Abdom-inal MRI confirmed bilateral cystic hemorrhagic adrenal lesions, while a mass of similar imaging characteristics (5 x 4 cm) at the lower pole of the left kidney was visible. The diagnosis was acute adrenal insufficiency due to secondary metastases. After hydration and replacement therapy with hydrocortisone and fludrocortisone symptoms gradually improved. A brain and neck CT as well as a bone scan did not reveal any other pathological findings consistent with malignancy. Biochemical cancer markers were negative too. A biopsy of the lesion at the lower pole of the left kidney under CT guidance was performed and resulted in findings consistent with sarcomatoid renal cell carcinoma. Thus, the patient underwent left radical nephrectomy-adrenalectomy. The histopathology examina-tion confirmed the diagnosis of sarcomatoid renal cell carcinoma.

Discussion: Adrenal incidentalomas are most commonly unilater-al. Nevertheless, bilateral adrenal incidentalomas are reported in 7.8-15% of cases. Most of them represent single discrete bilateral lesions, while in a small percentage appear as diffuse adrenal hy-perplasia or enlargement. The majority of lesions are benign and non-functional. However, the possibility of congenital adrenal hy-perplasia, metastases, hemorrhage, pheochromocytoma, lympho-

ma, infectious or metabolic storage diseases is higher compared to unilateral lesions. Acute adrenal insufficiency due to adrenal metastases is uncommon, despite the fact that adrenal glands rep-resent a common metastasis site for several solid tumors. This is likely due to the sufficient functional compensation by the adrenal glands and the fact that the signs and symptoms of deficiency are non-specific and often masked by the symptoms of neoplastic dis-ease. Renal cell carcinomas can metastasize to the adrenal glands via both the lymphatic and the venous system, but bilateral presen-tation of adrenal metastases is extremely rare. The optimal strate-gy for the detection of adrenal metastases including the possibility of biopsy as well the best treatment option are not clear in the lit-erature and should be individualized. The diagnosis and treatment approach needs multidisciplinary team collaboration with active participation of endocrinologists.

Keywords: adrenal, incidentalomas, bilateral, metastases

Figure 1: Chest VT: arrows indicate bilateral adrenal

lesions revealed incidentally

figure 1. Chest VT: arrows indicate bilateral adrenal lesions re-vealed incidentally

O – 26 adrenal

Bilateral CystiC HemOrrHagiC adrenal inCidentalOmas

STAVROULA A. PASCHOU1, KONSTANTINOS TzIORAS1, CHRISTINA NIKA1, ATHANASIOS PAPATHEODOROU2, VASSILIS SAMARAS3, ANDROMACHI VRyONIDOU1

1 Department of enDocrInology anD DIabetes, HellenIc reD cross HospItal, atHens, greece 2 Department of raDIology, HellenIc reD cross HospItal, atHens, greece 3 Department of patHology, HellenIc reD cross HospItal, atHens, greece

2016


Introduction: Primary adrenal cortical carcinoma is a high-grade tumor associated with poor prognosis. Sixty percent of adrenal car-cinomas are functional. Thirty percent of these patients may exhibit Cushing’s syndrome. This is the first case report in the literature of an adrenal carcinoma presenting with Cushing’s syndrome devel-oping on 8 year history of adrenomyolipomas and adrenal insuffi-ciency.

Case: A fifty years old male patient was admitted with fatigue and hyperpigmentation to another instution in 2008, when he was diag-nosed with adrenal insufficiency. At the time of diagnosis, abdomi-nal MRI revealed multiple lesions 2 cm mass on left and 5 cm mass in right adrenal glands consistent with adrenomyolipomas. He was not investigated further on the etiology of adrenal insufficiency, and his symptoms were alleviated with prednisolone and fludro-cortisone. In february 2016, patient was admitted to another ins-tutions’ Endocrinology outpatient clinic with hypertension, pretibial oedema, weight gain and weakness in lower extremities. He was started on antihypertensive medication. Routine laboratory tests were within normal limits. PET-CT revealed an increase in size of adrenal tumors; right adrenal mass was increased to 13 cm (SUV-max: 17.75) and left adrenal mass was increased to 5 cm (SUVmax: 14.47). Patient was admitted to our clinic for second opinion. In his physical examination, his face was plethoric, he had 3+ pretibial oedema bilaterally. His blood pressure was 160/90 mmHg. Routine laboratory tests revealed hypokalemia [3.1 mEg/L (3.5-5.1 mEq/L)],

proteinuria (1.700 mg/day) and increased androstenedione levels [264 ng/dL (40-150 ng/dL)]. fludrocortisone and prednisolone were gradually decreased and stopped. Basal cortisol levels were within normal levels even when the prednisolone was stopped (18.2 µg/dL). One-mg dexamethasone test did not suppress serum cortisol (13.3 µg/dL). Midnight cortisol was elevated (12.2 µg/dL). Salivary cortisol [0.91 µg/dL (0-0.41 µg/dL)] and urinary free cortisol levels were elevated as well [1207 nmol/day (36-137 nmol/day)]. ACTH levels were normal [8.76 pg/mL (0-46 pg/mL)]. Patient was diag-nosed as Cushing’s syndrome. Patient underwent bilateral adre-nalectomy, pathology revealed metastatic adrenocortical carcino-ma on right adrenal gland (ki-67: %10, 2 paraaortic lymph nodes were positive for metastases) and adrenomyolipoma on left adre-nal gland. Postoperatively, prednisolone and fludrocortisone doses were regulated, patient was consulted with oncology; mitotane was started.

Discussion: There are case reports available in the literature on adrenomyolipomas causing Cushing’s syndrome; however adrenal insufficiency has not been previously reported. There are no case reports of an adrenal carcinoma developing on adrenomyolipomas. This is a rare and interesting case report of an adrenal carcinoma presenting with Cushing’s syndrome developing on 8 year history of adrenomyolipomas and adrenal insufficiency.

Keywords: adrenocortical carcinoma, adrenal insufficiency, adre-

nomyolipoma

O – 27 adrenal

xCadrenOCOrtiCal CarCinOma Presenting WitH CusHing’s syndrOme in a Patient WitH 8 years HistOry Of adrenal insuffiCienCy and

adrenOmyOliPOmasTAHiR SAyGIN ÖğüT1, GÖKHAN TAzEGüL1, HüMEyRA BOzOğLAN2, ÖzLEM DOğAN2, NUSRET

yILMAz2, RAMAzAN SARI2, HASAN ALi ALTUNBAş2, iNANç ELif GüRER3, GüLTEKiN SüLEyMANLAR4, MUSTAfA KEMAL BALCI2

1 akDenIz UnIversIty, facUlty of meDIcIne, Department of Internal meDIcIne, antalya/tUrkey 2 akDenIz UnIversIty scHool of meDIcIne, Department of enDocrInology, antalya/tUrkey

3 akDenIz UnIversIty scHool of meDIcIne, Department of patHology, antalya/tUrkey 4 akDenIz UnIversIty scHool of meDIcIne, Department of nepHrology, antalya/tUrkey

2016


Granulomatous hypophysitis (GH) is a rare disease that mimics non-functioning pituitary adenomas. At magnetic resonance im-aging (MRI), the most common feature is pituitary enlargement. Histological examination is the gold standard for the diagnosis of GH. It is characterized by variable numbers of multinucleated giant cells and lymphocytes, numerous histiocytes, variable amounts of granuloma formations and fibrosis. Its most common presenting symptom is headache. In the absence of a demonstrable causative agent, such as; tuberculosis, sarcoidosis or Langerhans cell his-tiocytosis, it is termed as idiopathic granulomatous hypophysitis (IGH). Herein, we present a case with a macroadenoma filling the pituitary fossa that was diagnosed as IGH following surgery.

A 44-year-old woman was admitted to the hospital with a 5-months history of headache and oligomenorrhea. Magnetic resonance im-aging showed a macroadenoma with suprasellar extension abut-ting the optic chiasm and the pituitary stalk was deviated to the right. Her visual field test was normal. Hormonal profile demon-strated the followings: TSH:0.917 uIU/ml (0.4-4.6), freeT4:20.66 pmol/L (11.5-22.7), early morning cortisol:17.75 mcg/dl (4.3-22.4), fSH:19.11 mIU/mL (1.42-15.4), LH:8.5mIU/mL (1.24-7.8), Estra-diol: 19.85 pg/ml (97-216 pg/ml) and IGf-1:149 ng/mL (119-307). Her prolactin level was normal: 24.6 ng/mL (2.8-29.2). Based on the laboratory and MRI findings, the initial presumptive diagnosis of our case was a non-functioning pituitary macroadenoma and she underwent transnasal-transsphenoidal pituitary surgery. Her pathologic findings suggested granulomatous hypophysitis. It ex-hibited granulomatous changes with multinucleated giant cells and focal areas of necrosis. ziehl-Nielson (zN/AfB) and periodic acid Schiff’s (PAS) stains were done and demonstrated no evidence of acid fast bacilli or fungal elements. Once the histological find-

ings was definitive, further diagnostic work-up for the exclusion of systemic granulomatous diseases was performed, including; polymerase chain reaction (PCR) for Mycobacterium tuberculosis in tissue sample, purified protein derivated (PPD) test and comput-ed tomography (CT) of the thorax. Measurement of serum angio-tensin converting enzyme (ACE) and ophthalmologic examination were done for ruling out sarcoidosis. All the tests were found to be negative. Additionally, any autoimmune background was found. De-pending on the findings, the final diagnosis of our patient was IGH.

Idiopathic granulomatous hypophysitis is a rare inflammatory dis-ease of unknown etiology. It‘s not clear whether lymphocytic hy-pophysitis and IGH are separate diseases or are distinct presen-tations of the same disease. There is also some evidence in the literature that IGH is an autoimmune pituitary disorder. It is diffi-cult to diagnose IGH preoperatively and is often misdiagnosed. Al-though rare, it should be kept in mind in the differential diagnosis of sellar lesions.

Keywords: Hypophysitis, Granulomatous, adenoma

Figure 1: Histological photomicrographs

Granuloma with multinucleated giant cell (arrow), H&E x200 (fig-ure a), H&E x400 (figure b) Histiocytes showing positive CD68 with immunohistochemical staining x 200 (figure c)

O – 28 Pituitary

idiOPatHiC granulOmatOus HyPOPHysitis mimiCking Pituitary adenOma

GüLAy şiMşEK BAğIR1, fiLiz EKşi HAyDARDEDEOğLU1, OKAN BAKINER1, EMRE BOzKIRLI1, ÖzGüR KARDES2, fAziLET KAyASELçUK3, MELEK EDA ERTÖRER1

1 Department of enDocrInology, baskent UnIversIty, aDana,tUrkey 2 Department of neUrologIcal sUrgery, baskent UnIversIty, aDana, tUrkey

3 Department of patHology, baskent UnIversIty, aDana, tUrkey

2016


This case illustrates progress of a giant prolactin-secreting pitu-itary adenoma in young man with a family history of prolactinomas in the absence of the adequate therapy for 8 years after initial di-agnosis.

The 36 year-old Caucasian man admitted with complaints of in-tense headache and progressive visual deterioration. These com-plaints started at age 26 and MRI revealed pituitary macroadenoma at a size of 44*44*29 mm with severe extrasellar extension with chiasmal compression and involving right cavernous sinus. His se-rum prolactin level was 202.8 μg/L (4.9-25.4). The patient refused offered treatment via transcranial surgery with subsequent radio-therapy. further, the patient did not seek any medical treatment for 8 years. Increase the intensity of the headache prompted patient to repeat MRI which revealed continued tumor grow to 65*52*70 mm with dislocation of brain median structures with 5 mm.

Goldmann perimetry showed bitemporal hemianopsia. Accord-ing to laboratory analysis serum prolactin level in probe with di-lution was 17090.0 μg/L (4.0-15.2). Also there was sign of latent

secondary adrenal insufficiency, hypogonadotropic hypogonadism and secondary hypothyroidism. Cabergolin therapy with a gradual increase of dose up to 1.0 mg per week was initiated as well as replacement therapy with L-thyroxine.

After 2 months serum prolactin decreased to 10849.0 μg/L (4.0-15.2) and cabergoline dose was increased to 2.0 mg per week. Six month after initiation of treatment with cabergoline patient noted a regression of headache and improvement of visual function, serum prolactin level decreased by 36 times and reached 469.4 μg/L (4.0-15.2), tumor shrinkage reached 25%.

Described case emphasize the crucial role of identification of hy-perprolactinemia among young men on early stages of the disease and necessity of the proper treatment by dopamine agonists after verification of diagnosis. Cabergoline therapy is effective even in case of giant prolactinomas.

Keywords: giant prolactinoma, invasive tumor, panhypopituitar-

ism, cabergoline.

O – 29 Pituitary

Case Of giant PrOlaCtinOma in yOung manALEXANDR TSIBERKIN, TATIANA KARONOVA, ANNA DALMATOVA, NATALIA KLyAUS,

ELENA GRINEVAInstItUte of enDocrInology, feDeral almazov nortH-West meDIcal researcH centre, saInt petersbUrg, rUssIa

2016


Case: A 36 year old woman was admitted to our hospital with the complaint of fatigue. She had the story of medical follow-up at an-other hospital for 10 years because of pituitary insufficiency. She was taking hormonal replacement therapy for hypocorticolism, hypothyroidism and primary amenorrhea since she was a child. She experienced pubertal changes and had regular menstruation under cyclic estrogen/progesteron replacement. Detailed histo-ry revealed that the patient was born by vaginal delivery after full term pregnancy period. At birth, her weight and height were nor-mal, 3600 grams and 52 cm, respectively. There was no history of consanguinity between her parents.

She had no spesific complaint on admittance. Height was 157 cm, and weight was 54.4 kg. Her breasts were at Tanner 3 stage, axillar and pubic hair were scarce. Her laboratory examination revealed low IGf-1; 26.7 ng/ml (109-284), elevated prolactin; 54.86 ng/ml (2.8-29.2), suppressed TSH 0.01 UIU/ml with normal fT4;17.89 pmol/L (11.5-22.7) and low morning cortisol; 0.5 µg/dl (4.30-22.40). Since the patient was using estrogen/progesteron, gonadal axis was not evaluated. On pituitary MRI, the stalk was not observed before and after contrast administration and neurohypophysis was found to be located ectopically. According to clinical and radiolog-ical results, the patient was diagnosed as pituitary stalk interrup-tion syndrome.

Because of the high conccurance rate of malformations especially in the central nervous system and craniofacial structures, cranial MRI was performed and cerebellar tonsiller ectopia was reported besides ectopic neurohypophysis and absence of pituitary stalk.

figure 1. Pituitary images in coronal plane

figure 2: Pituitary images in saggital plane

Discussion: Pituitary stalk interruption syndrome is a rare con-genital defect and the cause has not been fully understood. The diagnosis depends on MRI findings. This syndrome causes vary-ing degrees of anterior pituitary hormone deficiencies and relat-ed complaints. Hypoglycemia during neonatal period, growth re-tardation and lack of pubertal development with age are the main contributors of clinical presentation. Lifetime hormonal replace-ment therapy is required in these patients. The MRI findings of pi-

O – 30 Pituitary

Pituitary stalk interruPtiOn syndrOme: a rare Cause Of PanHyPOPituitarism

fiLiz EKşi HAyDARDEDEOğLU, GüLAy şiMşEK BAğIR, OKAN SEfA BAKINER, EMRE BOzKIRLI, MELEK EDA ERTÖRER

Department of enDocrInology, baskent ünIversIty, aDana,tUrkey

2016


tuitary stalk interruption syndrome should fully indicate pituitary hormonal evaluation. Although the presence of malformation does not seem to be associated with severity of hormonal deficiencies,

these patients should be evaluated for the extrahypophyseal mal-formations, as well.

Keywords: Pituitary, stalk, interruption, panhypopituitarism

2016


Background: Insulinomas can be multifocal and each insulinoma may arise at different times in cases with MEN-1. Therefore, local-ization studies and treatment may fail.

Case Presentation: The patient was a 27 years old female, admit-ted for dizziness, occasional palpitation, feel of hunger, sweating, and headache, occurring during fasting. Laboratory findings re-vealed a fasting plasma glucose (fPG) of 47 mg/dL, insulin of 17.9 mIU/mL, C-peptide of 2.85 ng/mL, Ca of 12.1 mg/dL, P of 2.24 mg/dL, PTH of 272 pg/mL and 25 OHD3 of 4 ng/m L. Plasma glucose, serum insulin and C-peptide levels were 36 mg/dL, 15.4 mIU/mL and 2.82 ng/mL, at the time of symptomatic hypoglycemia during prolonged fasting test, respectively. A pituitary adenoma was found on MRI. A diagnosis of MEN-1 disease was made. She was com-menced on therapy with diazoxide 100 mg bd.

Imaging Studies: Abdominal ultrasonography (USG) and MRI were normal. Endoscopic ultrasonography (EUS) revealed a 9 mm hy-poecoic area in the tail of pancreas (figure 1). The result of fine needle aspiration biopsy of the lesion was consistent with pNET (figure 4). Parathyroid Tc-99m pertechnetate sestamibi/SPECT CT revealed radiotracer uptake at the level of C7 vertebrae.

Treatment, Outcome, and Follow-Up:On July 2010 left upper, lower and right upper glands parathyroidectomy with transcervi-cal thymectomy was performed. However, the right lower parathy-roid gland could not be found. Histopathological analysis revealed a parathyroid adenoma inside the thymus. A distal pancreatecto-my was performed on October 2010. Histopathological evaluation demonstrated 3 distinct well differentiated pNETs (WHO grade I). The largest tumor was 8 mm in diameter and was immunostained positively for glucagon and the other two tumors were 6 and 5 mm in diameters and both were immunostained positively for insulin (figure 5,6). On postoperative day 1, the serum Ca, P and PTH lev-els were 10.6 mg/dL, 2.7 mg/dL and 37.4 pg/mL, respectively. fPG levels was > 80 mg/dL immediatly after surgery.

On October 2011, she was admitted again for neuroglycopenic symptoms. Serum insulin and C-peptide levels were 8 mIU/mL and 1.54 ng/mL while fPG was 38 mg/dL on supervised fasting test.

She was commenced again on therapy with diazoxide 100 mg bd, pending imaging results. However, the results of abdominal USG, MRI, EUS and octreoscan, were unremarkable. The follow-up bio-chemical analysis results is presented in Table 1.The patient re-fused surgery.

On January 2016 she developed symptomatic hypoglycemia be-cause she stopped diazoxide treatment. Imaging studies includ-ing EUS could not show a pNET. 68Ga DOTATATE PET/CT (figure 7) showed three focal radio tracer uptakes in the head, body and tail of the pancreas. She developed severe pretibial edema enough to cause difficulty in walking. Therefore, the dose of diazoxide was reduced to 100 mg/day. Now, what kind of surgery should be pre-ferred in this case? enucleation of the tumors or a pancreatectomy?

Keywords: Multifocal insulinomas, Glucagonoma, 68Ga DOTATE/PET CT, endoscopic ultrasonography

figure 1

figure 1. Tc99m SPECT CT showing a parathyroid adenoma located in the thymus.

O – 31 neuroendocrine tumors

HOW multifOCal insulinOmas Can Be diagnOsed and treated in a Case WitH men-1 disease?

SAyiD zUHUR1, iDRiS KUzU2, yüKSEL ALTUNTAş2

1 Department of enDocrInology anD metabolIsm, namIk kemal UnIversIty facUlty of meDIcIne, tekIrDag, tUrkey 2 Department of enDocrInology anD metabolIsm, sIslI etfal traInIng anD researcH HospItal, IstanbUl, tUrkey

2016


figure 2. Head MRI Showing a pituitary adenoma of 4x4 mm

figure 3. Endoscopic Ultrasonography showing a 9 mm hypoechoic tumor in the tale of pancreas

figure 4.fine needle aspiration biopsy showing dens neuroendo-crine cells

figure 5. Immunocytochemistry showing two insulinomas.

figure 6. Immunocytochemistry showing a glucagonoma

figure 7. 68Ga DOTATATE PET/CT showing a tumor in the head of pancreas

2016


figure 8. Ga68 DOTATATE PET/CT showing two tumors in the body and tale of the pancreas

Table 1

2010 2010(Post-op) 2011 2012 2014 2015 2016

fPG (mg/dL) 47 80 72 76 66 71 42

Insulin (µIU/mL) 17.9 6.92 15.4 15.1 20 12

C-peptide (ng/mL) 2.85 1.86 2.33 2.74 3.51 2.54

iPTH (pg/mL) (<65) 272 37.4 14.2 120 62 66 70.8

Ca (mg/dL) 12 10.06 11.19 11.5 10.08 9.79 9.94

P (mg/dL) 2.24 2.79 1.83 2.3 2.9 2.9 3.3

25OHD3 (ng/mL) 4 25 24.17 27 27.2

Gastrin (pg/mL) (<115) 50.02 39.8

Urinary 5-HIAA (mg/24h) 4.7

Lon term follow-up laboratory results of the patient

2016


Introduction: Thymic neuroendocrine tumors are rare and they can be complicated by endocrine abnormalities. Although overall survival rates are poor, cure is possible with complete surgical re-section.

Case Presentation: 30 year old male was admitted to hospital with sudden onset chest pain, shortness of breath and fever. Diagnosis of pericardial effusion was made and the patient was transferred to our center’s cardiovascular surgery department for further evalua-tion. Laboratory workup was unremarkable except mild leukocyto-sis and elevated transaminases. Haemorragic effusion was drained by pericardiosynthesis. No malignant cells were seen on cytologic exam, tuberculosis culture was negative, as well as viral and reu-matologic markers. To visualize thoracic cavity and adjacent struc-tures, cardiac MRI was performed and 38x25x37 cm mass located to anterior mediastinum which was suggestive of thymic hyperpla-sia was detected. Surgical treatment recommendation was refused by the patient. One year later, the mass was enlarged to 45x35x65 mm, surgery was suggested again but the patient did not accept the operation since he had no complaints.

At the age of 35, he was admitted to general surgery clinic for swelling and compression feeling on his neck. Large thyroid nod-ules were seen at US and he was diagnosed as PTC by total thy-roidectomy. Several days after discharge, the patient came back with chest pain, dyspnea, ortopnea and edema. CT determined 110x54x38 mm thymic mass which was invasive to vena cava su-perior, possible metastatic nodules in the lung, mediastinal and

abdominal enlarged lymph nodes. He was hospitalized for worsen-ing symptoms plus emerging severe muscle weakness. Laboratory work-up revealed deep hypokalemia, mild leukocytosis and new onset diabetes. Due to his medical history of PTC, endocrinology consultation was done. The patient was hypertensive, ortopneic, had dark skin color with acne formations. These physical and labo-ratory findings were suggestive of ectopic Cushing’s syndrome. He had no family history of MEN syndromes. Serum ACTH and cortisol levels were 450 pg/mL and 102 µg/dL, respectively, while 24 hour UfC level was 4286 mcg/d. Biopsy of the thymic mass resulted as high grade neuroendocrine carcinoma. During hospitalization, thrombosis occured in the right cephalic vein and antibiotic therapy was started for hospital acquired pulmonary infection. Depending on the catastrophic Cushing’s syndrome’s life-threatening results, he was given to bilateral adrenalectomy. Twelve days after surgery, the patient died as a result of pneumonia.

Discussion: Although neuroendocrine ACTH-producing tumors of the thymus usually present with signs and symptoms of Cushing’s syndrome, our patient was known to have thymic mass for five years, but had ectopic Cushing’s syndrome only at late term, when the tumor was metastatic. Bilateral adrenalectomy was performed to save the patient from catastrophic Cushing’s, although he died of severe infection.

Keywords: Ectopic Cushing’s syndrome, catastrophic Cushing’s syndrome, thymic neuroendocrine neoplasia.


CatastrOPHiC CusHing’s syndrOme: rePOrt Of a mOrtal Case

SEDA OğUz1, CENK yüCEL BiLEN2, CENK SÖKMENSüER3, TOMRiS ERBAş1, SELçUK DAğDELEN1

1 Department of enDocrInology anD metabolIsm, Hacettepe UnIversIty meDIcal scHool, ankara, tUrkey 2 Department of Urology, Hacettepe UnIversIty meDIcal scHool, ankara, tUrkey

3 Department of patHology, Hacettepe UnIversIty meDIcal scHool, ankara, tUrkey

2016


A 36 year old female patient visited endocrinology outpatient clinic for type 2 DM. She had DM for 10 years, hypertension, rheumatoid arthritis, and thyroidectomy for multinodular goiter. She was on insulin therapy for 7 years. Her mother also had type 2 DM. She denied chronic steroid usage. She did not have any pancreatitis attack. She was on 50 U/day glargine insulin, 10 U tid aspart insulin, perindopril 5 mg qd, amlodipin 5 mg qd, levothyroxine 112.5 mcg qd therapy. She was not using met-formin due to adverse effects. She weighed 70 kg and had height of 149 cm. Physical examination was normal despite obesity. Acanthosis was absent. Then she developed CML (chronic myeloid leukemia). first, she was treated with hydroxyurea 500 mg tid and then medical therapy for CML was switched to imatinib. Although she had been on high dose in-sulin therapy, insulin requirement decreased dramatically to 16 U/day glargine insulin soon after imatinib therapy. Pioglitazone was added to the therapy in order to counteract insulin resistance. Two months after pioglitazone therapy glargin dose decreased to 12 U/d.

Causes of decreased insulin requirement are liver/renal failure, weight loss, decreased appetite, cortisol deficiency, growth hor-mone deficiency, hypothyroidism, and drugs (non-selective beta blockers, ACEI). Tyrosine kinase inhibitors (imatinib, dasatinib, and sunitinib) are indicated for treatment of metastatic thyroid cancer, CML, and gastrointestinal stromal tumours. Reduced insulin resis-tance and increased β-cell mass are the proposed mechanisms for improved glycaemia.

Since she did not have symptoms of primary/secondary cortisol defi-ciency (malaise, weakness, decreased appetite, hyperpigmentation, nausea-vomiting, diarrhea), cortisol and ACTH tests were not ordered. She did not have head trauma suggesting hypopituitarism. Since growth hormone deficiency leads to hypoglycaemia more frequently in children compared to adults without other accompanying hypophyseal hormone deficiencies, IGf-1 was not studied. Hypoglycaemia incidents decreased with decrements in daily insulin dose. Therefore we did not suspect an insulinoma. Her weight was almost stable and liver and renal function tests were normal. Her appetite was good. Near-normal TSH level ex-cluded hypothyroidism as a possible cause for hypoglycaemia and de-creased insulin demand. Perindopril may enhance hypoglycaemic ef-fects of anti-diabetic drugs and lead to hypoglycaemia in less than 1% of treated patients. Our patient was already on perindopril therapy, so we did not assume it as a contributing factor. We did not evaluate C-peptide and insulin levels regarding the fact that her ongoing insulin therapy would affect assessment of the results.

Cancers are usually known as “hyperglycaemic disease states” Hy-poglycaemia in a cancerous patient even in case of accompanying DM, should not be overlooked and simply assumed to result from poor intake and decreased demand for anti-diabetic medication

Keywords: imatinib, diabetes mellitus, tyrosine kinase inhibitors, hypoglycemia

O – 33 diabetes

effeCt Of imatiniB On insulin tHeraPy in a Patient WitH tyPe 2 dm

BENGüR TAşKIRAN1, GüVEN BARIş CANSU1, MUSTAfA KARAGüLLE2, iNCi VETEM KARAGüLLE3

1 Deparment of enDcorInology, yUnUs emre state HospItal, eskIseHIr, tUrkey 2 Deparment of Haematology, yUnUs emre state HospItal, eskIseHIr, tUrkey

3 Deparment of Internal meDIcIne, yUnUs emre state HospItal, eskIseHIr, tUrkey

Table 103/2012 10/2015 11/2015 12/2015 01/2016 02/2016 06/2016

glucose (mg/dl) 217 353 180 127 173 196 138creatinine (mg/dl) 0.68 0.75 0.58 0.70 0.69 0.72 0.65ALT (U/l) 16 18 19 10 10 10 11HgbA1c (%) 7.17 7.4WBC (/mm3) 10440 40550 13050 5330 6650 5640 5360Neu (mm3) 7930 34070 10410 3270 4270 3390 2870Hgb (g/dl) 11.1 13.1 12.2 11.2 11.6 12.3 11.8Hct (%) 32.8 38.6 36.2 33.7 35.0 36.6 34.2TSH (mIU/l) 5.49 5.48 4.31vit B12 (pg/ml) 383 153ferritin (ng/ml) 48.1 62.7ESR (mm/hr) 95 22 56 85 49 38 44total protein (g/dl) 7.7 6.7 6.9 6.6 6.8 7.0 6.92albumin (g/dl) 4.3 3.6 4.0 3.8 4.2 4.3 4.3HbsAg negative negativeAntiHCV negative negativelaboratory data before and during imatinib therapy

2016


Introduction: Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited multisystem familial tumor syndrome predis-posing to various benign or malignant tumors such as central ner-vous system and retinal hemangioblastomas, renal cell carcinoma and cysts, pancreatic tumors and cysts, pheochromocytomas, and endolymphatic sac tumors.

Case: 40-year-old woman was referred to our clinic with bilateral adrenal masses diagnosed at abdominal CT which was performed for lumbar pain. Her medical history was normal. There was no history of hypertension or hypertensive attack. But her brother had a history of bilateral adrenalectomy at 30-years-old. There wasn’t any abnormal finding in her physical examination. Her blood pres-sure was 120/80 mm/Hg and pulse rate was 72 beats per minute with a regular rhythm. In the abdomen CT there was a 4 cm nodular lesion on the left adrenal gland and a 2.5 cm nodular lesion on the right adrenal gland. Also there was a 2.5 cm nodular lesion around celiac trunk and a 3 cm nodular lesion at the neighborhood of pan-creas head. 24-hour urinary fractionated normetanephrine level was about 20 times higher than the normal range (8577 µg/24h, normal value: 88-444 µg/24h) and 24-hour urinary metanephrine level was 157 µg/24h (normal value: 852-341 µg/24h). Plasma normetanephrine level was also found increased (14.88 nmol/L, normal value: 0.12-1.18) while metanephrine level is normal. Af-ter the diagnosis of pheochromocytoma, she was operated fol-lowing appropriate medical preparation. Pathological examination

of the bilateral adrenalectomy material was pheochromocytoma, paraaortic mass excision was paraganglioma and the pancreatic nodular lesion was neuroendocrine tumor. She was screened clini-cally for multiple endocrine neoplasia (MEN) type 2A and there was no evidence for primary hyperparathyroidism and medullary thy-roid carcinoma. We evaluated our patient for VHL disease because of the young age at presentation, presence of family history for the pheochromocytoma, coexistence of bilateral pheochromocytoma, abdominal paraganglioma and pancreatic neuroendocrine tumor. Central nervous system hemangioblastomas, renal cell carcino-mas or renal cysts were not detected. Genetic screening was also made. Gene mutation for the VHL disease found positive.

Conclusion: Pheochromocytomas are seen both sporadically and in association with a number of genetic syndromes, including VHL disease, MEN 2, neurofibromatosis type 1, and mutations of the succinatede hydrogenase subunits B, D, and C. VHL disease is a rare hereditary syndrome characterized by benign or malig-nant tumors. Pheochromocytomas in VHL disease tend to be seen in younger patients, are often bilateral, may be extraadrenal, and are less likely to be associated with symptoms of catecholamine production compared with those occurring in patients without VHL. Genetic screening should be considered for the patients with pheo-chromocytomas to proper treatment and the follow-up.

Keywords: pheochromocytoma, adrenal, genetic

O – 35 adrenal

a Case Of vOn HiPPel-lindau disease WitH Bilateral PHeOCHrOmOCytOma,

aBdOminal ParagangliOma and PanCreatiC neurOendOCrine tumOrSiNEM KIyICI1, BURçiN UyGUN1, SEMRA AyTüRK2

1 Department of Internal meDIcIne, saglIk bIlImlerI UnIversIty, yüksek İHtIsas eDUcatIon anD researcH HospItal, bUrsa, tUrkey

2 Department of enDocrInology anD metabolIsm, trakya UnIversIty meDIcal facUlty, eDIrne, tUrkey

2016


Introduction: Von Hippel-Lindau (VHL) disease is characterized by (1) hemangioblastomas involving the retina, cerebellum, brain stem, and spinal cord; and (2) visceral malignancies, such as renal cell carcinomas, pheochromocytomas, and pancreatic neuroendo-crine tumors. Here we present a case of VHL disease, who initially presented with mixed-type deafness and hypothyroidism and then diagnosed with VHL disease via familial screening.

Case Presentation: A 19-year-old woman was diagnosed with VHL-disease during familial screening, after her mother, the in-dex case, was also diagnosed with VHL-disease. Her mother had initially presented to the clinic with numbness in her left arm and during the course of her disease, she was diagnosed with cere-bellar hemangioma, renal cell carcinoma, bilateral pheochromocy-toma, and non-functional neuroendocrine tumor of the pancreas. Both she and her daughter were found to have heterozygous c.353 T>G mutation in the VHL gene.

Before her genetic diagnosis, she had first presented with delayed speech and was diagnosed with bilateral mixed type hearing loss when she was 3 years old. Later in the course of her routine out-patient follow-up, she was diagnosed with Hashimoto’s thyroiditis when she was 12 years old and levothyroxine replacement was started. After the patient was found to be heterozygous for the mu-tation, potential neoplasms were checked. She was normotensive and asymptomatic except for her hearing loss, and both physical and ophthalmological examinations were unremarkable. Cranial, spinal, and cochlear MRI revealed no abnormalities. After bilater-

al hypoechoic adrenal masses were found on the initial abdominal ultrasonography, abdominal MRI was performed and it confirmed a 17×15 mm right and a 29×26 mm left adrenal mass, both of which were hyperintense in T2A sequences and showed notable contrast uptake. Both the pancreas and the kidneys were normal.

further studies to delineate the nature of the adrenal masses were performed. 24-hour urine metanephrine, normetanephrine, and vanilmandelic acid levels were all in normal ranges and 68Ga-DO-TATATE PET imaging showed increased uptake at both adrenals. Based on the functional imaging results at VHL background, bi-lateral (silent) pheochromocytoma was diagnosed and bilateral adrenalectomy was performed. Immunohistochemistry showed negative staining for S100 and pan-CK staining and positive for syn-aptophysin and chromogranin, all of which were consistent with the diagnosis of pheochromocytoma.

Discussion: Genetic testing plays an important role for the work-up of the VHL disease. Here we presented an asymptomatic case of VHL-disease, diagnosed via genetic testing, and was found to have bilateral pheochromocytomas. Hearing loss can be seen with endolymphatic sac tumors associated with VHL disease, but crani-al and cochlear imaging revealed no abnormalities in this patient. A review of literature showed no reports of association between VHL-disease and hypothyroidism.

Keywords: von Hippel-Lindau disease, pheochromocytoma, hypo-thyroidism, hearing loss

0 – 36 adrenal

silent PHeOCHrOmOCytOma in vOn HiPPel-lindau disease: rePOrt Of a Case

RIzA CAN KARDAş1, SEDA OğUz2, AHMET BüLENT DOğRUL3, CENK SÖKMENSüER4, SELçUK DAğDELEN2

1 Hacettepe UnIversIty scHool of meDIcIne, Department of Internal meDIcIne, ankara, tUrkey 2 Hacettepe UnIversIty scHool of meDIcIne, Department of Internal meDIcIne, DIvIsIon of enDocrInology anD

metabolIsm , ankara, tUrkey 3 Hacettepe UnIversIty scHool of meDIcIne, Department of general sUrgery , ankara, tUrkey

4 Hacettepe UnIversIty scHool of meDIcIne, Department of patHology , ankara, tUrkey

2016


Adrenocortical carcinoma(ACC) is very rare tumors and much more uncommon with pregnancy. Only few case reports are reported in the literature because of the decreased fertility in patients with ACC in addition to rarity of the disease. We present the case of a 31- year-old pregnant woman with ACC.

Case: A 31-year-old woman in her second pregnancy was admitted to the hospital with sudden onset flank pain in her left upper quad-rant at 33 weeks’ gestation.

She was normotensive (125/75mmHg) and physical examination was normal.

Laboratory studies revealed Hb: 9,2 g%, Hct: 28,5%, PLT: 161000 /mm3, Glu: 129 mg/dL (random), Cr: 0.4 mg/dL, K:3.7 mmol/L, Na: 139 mmol/L, albumin: 3,2 gm/dL (3.5-5.5), LDH: 637 U/L (135-250). Abdominal ultrasonography and MRI revealed a large heteroge-neous tumor in the left adrenal gland measuring 145x100 mm. fetal development was normal as per gestational age. A detailed endocrine assessment was done. At the first week of hospitaliza-tion preterm labor began and the patient underwent cesarean sec-

tion. The female baby was healthy, weighing 2350 g, Apgar score 9/10. The patient was normtensive and normoglycemic during her stay in the hospital. 24-hour urine for metanephrines and normeta-nephrines were normal. Morning cortisol level measured was 21,6 mcg/dl (4-19,5), ACTH: 7,6 and 13,8 pg/mL. Low-dose dexametha-sone suppression test revealed cortisol level 7,9 mcg/dL. Testos-terone and DHEA-S levels were 0,608 ng/mL (0,05-0,52) and 202 μg/dL (35-430), respectively. 17-OH Progesterone was 15.8 ng/ml and TSH was 3,13 μIU/mL (0,2-4,2). The patient was evaluated by a multidisciplinary team and underwent to laparoscopic surgery. Pathological evaluation revealed, left adrenocortical carcinoma with a Ki score of 30%.

ACC can cause maternal and fetus mortality. Tumor size, Ki-67 in-dex, stage, and resection status are important prognostic indica-tors of survival in ACC patients. Prognosis of ACC during pregnancy is reported to be poorer than nonpregnant women. Hence early dis-ease recognition is essential.

Keywords: adrenocortical carcinoma, pregnancy, laparoscopy

0 – 37 adrenal

adrenOCOrtiCal CarCinOma in PregnanCySEHER TANRIKULU1, SAKiN TEKiN1, AyşE KUBAT üzüM1, NiHAT AKSAKAL2,

TUğBA SARAç SiVRiKOz3, SEMEN ÖNDER4, fERiHAN ARAL1

1 Department of enDocrInology, IstanbUl UnIversIty, IstanbUl, tUrkey 2 Department of general sUrgery, IstanbUl UnIversIty, IstanbUl, tUrkey

3 Department of gynecology anD obstetrIcs, IstanbUl UnIversIty, IstanbUl, tUrkey 4 Department of patHology, IstanbUl UnIversIty, IstanbUl, tUrkey

2016


Introduction: The adrenal gland is considered as an exceptional localization of a hydatid cyst. Adrenal hydatid cysts are usually sec-ondary to generalized echinococcosis. The reported incidence of adrenal hydatid cysts is 0.5%. We present here a case of an adrenal hydatid cyst mimicking a malignant tumour in the imaging tech-niques such as magnetic resonance imaging (MRI) and positron emission tomography (PET)- computerized tomography (CT).

Case: A 70-year-old woman was admitted to our hospital for ab-dominal pain. She had undergone surgery for liver hydatid cysts 4 years ago. No hypertension was present in medical history. In her physical examination; blood pressure, temperature, and heart rate were 130/90 mmHg, 36.9ºC, and 88/min, respectively. Tender-ness in the epigastrium and right hypochondrium were observed on palpation. Other physical examination findings were normal. Blood tests revealed leukocytes and eosinophils within normal values except anemia (Hb:11.2 gr/dL). Erythrocyte sedimentation rate was 93 mm/h. Abdominal ultrasonography revealed 71x91 mm and 61x65 mm hypoechoic masses containing internal cystic components in the liver. Abdominal MRI showed that there were cystic masses with 8 cm and 9 cm in diameter in the right lobe of the liver, and a 65x40 mm heterogenous probably malignant mass with internal cystic components in the right adrenal localisation which was showing diffusion limitation and contrast enhancement in early and delayed phases (figure 1). Indirect hemaglutination test of echinococcus granulosus was positive. Renin, aldosterone, plasma metanephrine and normetanephrine, fractionated urinary catecholamine and dehydroepiandrosterone sulfate levels were normal. One mg dexamethasone suppression test was evaluated as negative. In PET-CT, increased 2-[fluorine-18]-fluoro-2-de-oxy-D-glucose (fDG) involvement was observed in the right adre-nal mass (SUVmax 8.3), and cystic lesions of liver (SUVmax 4.8). She underwent an open laparotomy, and drainage of hepatic cysts

and right adrenalectomy were performed. Histopathological exam-ination revealed a cuticular layer and germinative membrane.

Conclusion: Hydatid cystic disease of the adrenal glands is rarely observed in clinical practice. Most hydatid cysts arising from the adrenal gland are part of a disseminated disease. These are most-ly asymptomatic and found incidentally by imaging or during sur-gery for other abdominal pathologies. Treatment of adrenal hydatid cysts is mostly surgical (pericystectomy or resection of the entire adrenal gland). The adrenal cyst hydatids can mimic malignant ad-renal lesions in imaging techniques as in our case. Adrenal hydatid cysts should be considered in patients with incidentally discovered adrenal masses especially with a history of hydatid cystic disease.

Keywords: adrenal gland, liver, hydatid cystic disease, imaging techniques

figure 1. Post-contrast T1-weighted axial MRI shows lesion con-taining heterogenous contrast accumulation

0 – 38 adrenal

a Case WitH adrenal Cyst Hydatid mimiCking malignant tumOur in mri and Pet-Ct

HüSNiyE BAşER1, OyA TOPALOğLU2, NESLiHAN çUHACI2, MEHMET KILIç3, NiüUfER POyRAz4, HiLAL AHSEN5, MELTEM yILDIRIM6, REyHAN ERSOy2, BEKiR çAKIR2

1 Department of enDocrInology anD metabolIsm, atatUrk eDUcatIon anD researcH HospItal, ankara, tUrkey 2 Department of enDocrInology anD metabolIsm, yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, ankara, tUrkey

3 Department of general sUrgery, yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, ankara, tUrkey 4 Department of nUclear meDIcIne, atatUrk eDUcatIon anD researcH HospItal, ankara, tUrkey

5 Department of patHology, atatUrk eDUcatIon anD researcH HospItal, ankara, tUrkey 6 Department of raDIology, yIlDIrIm beyazIt UnIversIty facUlty of meDIcIne, ankara, tUrkey

2016


Background: Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, sur-gery is often difficult and associated with considerable risks.

History: 10-year old girl presented with early menstrual cycles. The condition started at age of one year when her parents noticed that their child has developed abnormal vaginal bleeding. Her cy-cles were regular, each cycle lasted for 3 days. Her parents also noticed that she has developed breast, axillary and pubic hair at the age of five, and seven respectively. The parents also gave history of difficulty in speech especially articulation and abnormal general-ized body movements (epilepsy) since childhood for which she is on medications. She also had attacks of inappropriate laugh.

Diagnosis and Treatment: Her lab tests consisted with central precocious puberty, MRI shows suprasellar mass and she had been put on leuprolide and antiepileptic medicines until surgery planned. Her surgery was done with complete resection, with histopathology showing hamartoma. After surgery there was complete remission of seizure.

Conclusion: The treatment of hypothalamic hamartoma (HH) as-sociated with generalized epilepsy (GE) has been found to improve seizures and behavioral disturbances with an acceptable morbidity rate by using a variety of surgical approaches. Partial resection of tumor may be sufficient to reduce seizure frequency and to improve behavior and quality of life with few side effects.

Keywords: hypothamus, precocious puberty, hamartoma

O – 39 Pituitary

HamartOma Of HyPOtHalmus Presented as PreCOCiOus PuBerty and ePilePsy in 10-years

Old girlBAyAR AHMED QASIM

Department of meDIcIne, UnIversIty of DUHok, Iraq

2016


Introduction: Rosai Dorfman disease (RDD) is a benign non-Lang-erhans cell histiocytosis with multiorgan involvement. The connec-tion between histiocytosis and central diabetes insipidus (CDI) was first reported in the late 1800s, and it has been a rare cause of CDI since.

Case Presentation: 43-year old female was refered to our endo-crinology department because of polyuria and polydipsia. Water re-striction test was consistent with central diabetes insipidus. Labo-ratory tests showed no abnormality of anterior pituitary hormones. Desmopressin treatment was initiated. One year later she applied because of diplopia, fatigue, fever, night sweats and skin lesions. Skin lesions first started as periorbital pruritus and dryness, than expanded to perioral region, cheek, dorsum and chest in sever-al months. Physical examination revealed yellowish papules and plaques on periorbital region an bilateral cervical lymphadenop-athy. Punch biopsy of the skin lesions resulted as polymorphous infiltrate of lymphoid cells which were predominantly histiocytes. Exisional biopsy of the lymph nodes were reported to be infiltrat-ed with large histiocytes, lymphocytes, and plasma cells. Since the patient was diagnosed as CDI, pituitary MRI was performed. MRI detected normal pituitary gland thickness and signal intensity, dif-fuse enhancement in dynamic-contrast images, loss of the charac-teristic hyperintensity of the neurohypophysis, bilateral cavernous sinus infiltration and expansion, anterior epidural focal thickening below C3 level. Besides, sclerotic lesions were detected on thoracic vertebraes and sternum. The patient was diagnosed as Rosai Dorf-man disease with multi-system involvement, affected organs being neurohypophysis, bones and skin. Interferon alpha treatment was planned.

Discussion: Out of the 126 cases of RDD with CNS involvement in the literature, only 5 had CDI. So RDD should always be kept as a differential diagnosis of CDI with or without multi system involve-ment.

Keywords: Rosai Dorfman Disease, Histiocytosis, Central Diabe-tes Insipidus

Picture 1. facial lesions

O – 40 Pituitary

a rare Cause Of diaBetes insiPidus: rOsai dOrfman disease

ADiLE BEGüM BAHçECiOğLU1, ALPER GüRLEK2

1 Hacettepe UnIversIty scHool of meDIcIne, Department of Internal meDIcIne , ankara, tUrkey 2 Hacettepe UnIversIty scHool of meDIcIne, Department of Internal meDIcIne, DIvIsIon of enDocrInology anD

metabolIsm , ankara, tUrkey

2016


Introduction: Insulinomas are rare neuroendocrine tumors charac-terized by excessive insulin secretion. When these tumors come to clinical attention, they are usually small, measuring less than 2 cm in most of the cases. The only curative treatment modality is surgical resection of the tumor. Despite advances in imaging techniques, pre-operative localization of insulinomas may be challenging.

Case: A 52-year-old man was admitted to the endocrinology clinic due to recurrent episodes of confusion, blurred vision, diaphore-sis, and dizziness lasting for 6 months. Clinical symptoms were most likely to occur after a missed meal during the day. He had a weight gain of about 6 kg in the preceding 6 months. His pri-or medical history and family history were unremarkable. Physi-cal and neurological examinations were normal. He underwent a supervised 72-hour fasting test. After 10 hours of fasting, the pa-tient developed hypoglycemic symptoms and blood tests revealed a low plasma glucose (42 mg/dL) and abnormally elevated levels of insulin (40.4 µIU/mL) and c-peptide (3.13 ng/mL). Tumor local-ization studies including magnetic resonance imaging of the ab-domen and endoscopic ultrasonography were negative. In order

to localize the tumor, selective intraarterial calcium stimulation with hepatic venous sampling was performed. Calcium injection of proximal splenic artery caused more than 2-fold increase in hepat-ic insulin above baseline, indicating a lesion at the body/tail region of the pancreas. The patient underwent laparotomy. Careful man-ual palpation of the pancreas revealed a 1.5 cm nodular lesion in the tail. Distal pancreatectomy was performed. Histopathological findings were consistent with well-differentiated neuroendocrine tumor showing positive staining with insulin, synaptophysin, and chromogranin. Postoperatively, the patient was free of all previous symptoms and discharged with normal glycemic values.

Conclusion: Selective intraarterial calcium injection of the major pancreatic arteries with hepatic venous sampling is significantly superior than the non-invasive imaging modalities as a preoper-ative localizing study for insulinomas. Since it is an invasive study, must be reserved for the cases in which conventional non-invasive imaging methods do not localize the tumor.

Keywords: insulinoma, intraarterial calcium stimulation, magnet-ic resonance


lOCalizatiOn Of an insulinOma By seleCtive intraarterial CalCium stimulatiOn WitH

HePatiC venOus samPlingNAfiyE HELVACI, BüLENT OKAN yILDIz, GÖKHAN BAyKAL

Hacettepe UnIversIty scHool of meDIcIne, DIvIsIon of enDocrInology anD metabolIsm , ankara, tUrkey

2016


Introduction and Background: MEN type 1 is a rare, autosomal dominantly inherited syndrome presenting with various neoplasms of the parathyroid glands, pancreas and anterior pituitary. Many other endocrine and non-endocrine tumors also may complicate the clinical picture.

Clinical Case: We report the case of a 29-year-old male who pre-sented with an episode of hypoglycemia to an outside hospital. As the prolonged supervised fasting test was positive; insulinoma was clinically suspected. A subsequent abdominal magnetic resonance imaging (MRI) study revealed two focal pancreatic mass lesions lo-cating in the body and uncinate process of pancreas, measuring 22 mm and 13mm, respectively. Additional laboratory tests also re-vealed associating hypercalcemia and hyperprolactinemia. He was referred to our center with the preliminary diagnosis of MEN type 1. Primary hyperparathyroidism was diagnosed with clinical and lab-oratory findings and a subsequent pituitary MRI study depicted a macroadenoma with a size of 10x9x11 mm. With these findings, the

patient underwent total pancreaticoduodenectomy as the patient had a history of severe hypoglycemia. Pathological findings of the surgical specimen confirmed the clinical diagnosis of insulinoma by demonstrating multicentric well-differentiated foci of neuroen-docrine tumors. These tumors stained positive for insulin and syn-aptophysin as well as negative for glucagon, calcitonin, S100 and pan-keratin. Subtotal parathyroidectomy was also performed with the final diagnosis of parathyroid hyperplasia. As for hyperprolac-tinemia treatment, we started oral cabergoline.

Conclusion: MEN type 1 is a rare, incurable but managable syn-drome. Treatment plan depends on the patients’ dominant symp-tom. As the main symptom in our case was hypoglycemia, we elect-ed to proceed with treatment of the pancreatic neuroendocrine tumors before the removal of the parathyroid adenoma.

Keywords: MEN 1, insulinoma, hypoglycemia, pancreatic neuroen-docrine tumor, hyperparathyroidism

0 – 42 neuroendocrine tumors

knOWn syndrOme, a neW CaseMELTEM KOCA1, SEDA OğUz2, DERyA KARAKOç3, UğUR üNLüTüRK2

1 Department of Internal meDIcIne, Hacettepe UnIversIty, ankara, tUrkey 2 DIvIsIon of enDocrInology anD metabolIsm, Hacettepe UnIversIty, ankara, tUrkey

3 Department of general sUrgery, Hacettepe UnIversIty, ankara, tUrkey

2016


POSTERPRESENTATIONS

2016


Introduction: Autoimmune Polyglandular Syndromes (APS) are characterized by loss of immune tolerance to self-antigens leading to autoimmune destruction of endocrine glands and other tissues.

Clinical Case: We are presenting the case of 28 years old female patient. When the patient was 13 years old, she was referred to en-docrinology department because of increased pigmentation of skin and mucosal surfaces. Since physical and laboratory findings were compatible with adrenal insufficiency, she was started on hydro-cortisone treatment. The patient was also diagnosed Hashimoto’s thyroiditis concurrently with adrenal insufficiency. She had a fam-ily history of hypothyrodism and diabetes mellitus type 2 (mother and aunts). After six years she was admitted to our center with the complaints of polydipsia, polyuria and loss of weight. She was di-agnosed with type 1 diabetes mellitus. Since she had complaints

of abdominal pain and diarrhea, blood tests for Celiac disease an-tibodies were done, but was found negative and the colonoscopy was normal. Autoimmune thyroid disease, autoimmune adrenal insufficiency and type 1 diabetes mellitus supported the diagnosis of autoimmune polyglandular syndrome type 2.

Conclusion: APS type 2 is the most common of the endocrinopa-thy syndromes. APS is characterized by autoimmune adrenal in-sufficiency in combination with autoimmune thyroid disease and/or type 1 diabetes mellitus. APS type 2 is diagnosed primarily in adulthood, usually in third or forth decades of life and is extremely rare in childhood.

Keywords: Autoimmune adrenal insufficiency, diabetes mellitus type -1, Hashimoto’s thyroiditis

P – 01 adrenal

autOimmune POlyglandular syndrOme tyPe 2- in CHildHOOd

AfRUz MAMMADOVA, MiyASE BAyRAKTARDepartment of Internal meDIcIne, ankara, tUrkey

2016


Objective: Pheochromocytomas are rare and if missed they can expand devastating size. At this point bigger size can be a problem for surgery. To know the mass’ nature and anatomic localization clearly can help the clinician before the surgery and it can provide optimal perioperative care for the patient. Ga-68 DOTATE labeled somatostatin analogues to image neuroendocrine cancers has raised the interests to use for NETs. Ga 68-DOTATATE PET-CT has demonstrated superiority in lesion detection compared to scintig-raphy and MRI. Consequently usage of Ga-68 DOTATATE PET-CT for pheochromocytomas is a good option to demonstrate the mass and it should be kept in mind as a tool to clarify the exact localization and anatomy of the lesion.

Case: A 61-year-old male patient was admitted to surgery clinic complaining of stomach ache. After physical evaluation and routine blood tests surgeon performed computer tomography of abdomen. They found out adrenal mass and they referred the patient to our endocrinology outpatient clinic. We evaluated whether the tumor is hormone secreting or not. We checked the patients complaints in more detail and we realized that patient had hypertension, fatigue, palpitation and flushing. His normetanefrine was 1221 mg/24h and noradrenaline 318 mg/24/h. After biochemical evaluation patient had diagnosis of pheochromocytoma. We decided to perform surgi-cal intervention for the patient. But her adrenal mass had 10x12 cm in diameter. Also the patient had several comorbid problems like coronary heart disease and type 2 diabetes mellitus. Surgery would be very life threatening for the patient. After further evaluation of computer tomography we decided to use Gallium-68 Dotatate pos-itron emission tomography technic before surgery.

Conclusion: Our aim was to reveal the mass’ anatomic position and understand clearly the nature of the mass. Afer Gallium-68 Do-tatate Pet scanning we realized that the mass has hypometabolic region in the center. Also it had hypermetabolic region just only on the surface of the mass. (figure 1 ) After this information surgery became more feasible. In this case we wanted to emphasize that if a patient has an adrenal mass and surgery is life threating, Ga-68 Dotatate Pet scanning can be an useful technic to understand he nature of the mass clearly.

Keywords: GA-68 Dotatate scan, pheachromocytoma, Adrenal sur-gery

Figure 1

figure 1.Adrenal mass with necrosis in the center

P -02 adrenal

ga-68 dOtatate Can PrOvide surgery mOre feasiBle in Patient WitH devastating

PHeaCHrOmOCytOma mass.AyTEN ERAyDIN1, zEyNEL ABiDiN SAyINER1, UMUT ELBOğA2, SUzAN TABUR1, MESUT ÖzKAyA1,

ERSiN AKARSU1, MUSTAfA ARAz1

1 Department of enDocrInology anD metabolIsm, gazIantep UnIversIty, gazIantep, tUrkey 2 Department of nUclear meDIcIne, gazIantep UnIversIty HospItal, gazIantep, tUrkey.

2016


Introduction: Adrenal cystic lesions (CALs) are rare and form a heterogeneous group of lesions. Adrenal vascular cysts account for 84% of adrenal cysts. These lesions are more common in women and present clinically with abdominal pain or are found incidentally. Since their imaging features are not specific, endocrine evaluation should be performed carefully.

Case Presentation: 50-years-old woman was referred to our in-stitution from a clinic where she presented with right sided flank pain and abdominal ultrasound detected a nonspecific cystic le-sion at the upper region of the abdomen suggesting a liver cyst. An abdominal MRI showed a mass lesion of 43x22 mm at right ad-renal gland which was hyperintense in T2A sequences, containing calcified areas and showing marked contrast uptake. The charac-teristics of the lesion indicated preliminary diagnosis of pheochro-mocytoma or malignant adrenal mass rather than a benign lesion. The hormonal evaluation was completely normal. The mass was laparoscopically removed. Pathological examination revealed the diagnosis of vascular cyst with positive CD 34 and CD 31 staining.

Conclusion: Adrenal cysts are rare but important because of the clinical emergencies they may cause and the incidental malignan-cies or functioning tumors they may contain. Accordingly, all CALs require complete endocrinological evaluation.

Keywords: Adrenal vascular cyst, adrenal mass, vascular cyst,

figure 1. MRI

figure 2. MRI

P – 03 adrenal

adrenal vasCular Cyst: a rare fOrm Of adrenal mass

JAVANSHIR VAHABOV1, SEDA OğUz2, BüLENT OKAN yILDIz2

1 Department of Internal DIseases, Hacettepe UnIversIty, ankara, tUrkey 2 Department of Internal DIseases enDocrInology sUbDIvIsIon, Hacettepe UnIversIty, ankara, tUrkey

2016


Introduction: fahr’s syndrome is a rare neurological disorder characterized by abnormal calcified deposits in basal ganglia and cerebral cortex. Clinical manifestations are neuropsychiatric ab-normalities and movement disorders. There are various etiological factors. Biochemical abnormalities are nearly always absent and may worsen the course of the disease if untreated. We presented a patient with primary hypoparathyroidism and fahr’s syndrome who has resistant hyperphosphatemia.

Case: A 74-year old woman admitted to our clinic with involuntary movements and speech impairment. She had no disease or oper-ation previously, or no disease in her family history. Her physical examination was normal except chorea in right forearm, tremor in both hands with orofacial dyskinesia and slurred speech. Her blood test results were: Creatinine:0.76 mg/dl, Calcium (Ca):4.36 mg/dl, Phosphorus (P): 5.91 mg/dl, Magnesium (Mg): 1.79 mg/dl, Parathormone (PTH):1.5 pg/ml, 25-OH-Vitamin D3 (25-OH-D):<3 ng/ml. Primary hypoparathyroidism was diagnosed and she was hospitalized and treatment with parenteral calcium, and vitamin D replacement continued with oral calcium carbonate and calcitriol. To rule out cerebrovascular pathology, cranial computed tomog-raphy (CT) was performed and multiple bilateral symmetric calci-fications were assessed in basal ganglia, periventricular regions and both cerebellar hemispheres (figure1). This finding suggested fahr’s syndrome. Electroencephalography revealed pathological pattern which necessitated 1000 mg/day sodium valproate to start. Her neurological complaints did not change with recovery of calci-um level. Haloperidol was added to resolve the unintentional move-ments. She was discharged calling her for the next visit. Ca:9.1 mg/dl, P: 6.4 mg/dl, Mg: 1.9 mg/dl was retrieved after two weeks. After one week the result of Ca:8.5 mg/dl, P: 6.5 mg/dl directed us to re-hospitalize her to control her adherence to drug. Calcium citrate dosage increased up to 9000 mg/day throughout hospital stay with cessation of calcitriol and vit D3. This gave the results of Ca:8.5 mg/dl and P: 6.3 mg/dl. Disability of hyperphosphatemia management accompanied continuation of chorea and orofacial dyskinesia.

Conclusion: Hyperphosphatemia would accompany fahr’s syn-drome when the etiology is hypoparathyroidism. Seizures and movement disorders in fahr’s syndrome can be resolved with the correction of phosphate and calcium levels. Despite managing nor-mocalcemia, inability in normalization of phosphate levels would be one reason not to establish stabilizing neurological manifesta-tions of the syndrome.

Keywords: fahr’s syndrome, hypoparathyroidism, hyperphospha-temia

Figure 1

figure 1. Calcificatiton in basal ganglia

Figure 2

figure 2. Calcification in cerebellar hemispheres

P – 05 Bone/Calcium

a faHr’s syndrOme Patient WitH resistant HyPerPHOsPHatemia

iffET DAğDELEN DURANDepartment of enDocrInology anD metabolIc DIseases, kIrIkkale yüksek İHtIsas HospItal, kIrIkkale, tUrkey

2016


40-year-old male was referred to the diabetes clinic for help in managing his diabetes. Two years before his visit, diabetes was di-agnosed after history of osmotic symptoms of polyuria, polydypsia and weight loss. He was started on oral hypoglycaemic agents. He initially responded to this treatment, but over the next one year his medication doses were slowly raised until he was on 60mg of gli-clazide and 2,000 mg metformin. At the time of referral, his fasting blood glucose levels were in the range of 180 mg/dl and and his haemoglobin A1c (A1C) was 10%. He was started on basal insulin, insulin glargine.

His medical history was not significant for any alcohol intake or hepatitis. family history was negative for diabetes and hemochro-matosis. His review of systems was positive for joint discomfort in both hands along with 6 month history of decreased libido and erectile dysfunction.

Physical exam revealed normal vital signs and no retinopathy or other signs of diabetic complications. His hand joints showed mild swelling and tenderness over the proximal interphalangeal joints, and his skin was slightly, diffusely hyperpigmented.

Lab data included a random glucose of 253 mg/dl, A1C of 10%, normal creatinine and electrolytes, aspartate aminotransferase (SGOT) of 77 IU/L (normal < 44 IU/L), alanine aminotransferase (ALT) of 144 IU/L (normal < 31 IU/L ), normal bilirubin and alkaline phosphatase levels, low testosterone level 9 nmol/l (normal range

12-30 nmol/l), and negative hepatitis antigen screen. His iron level was 386 μg/dl (normal < 155 μg/dl) with an iron-binding capacity of 296 μg/dl (normal < 400) and percent transferrin saturation of 97% (normal < 50%). Serum ferritin was 3028 μg/l (normal < 160 μg/l). Screen for autoimmune hepatitis was negative. Genetic testing demonstrated homozygosity for the C282y chromosome. Referral to the hepatology clinic was done and liver biopsy was carried out, which identified increased iron stores and early periportal fibrosis.

A detailed review of the liver functions done prior to onset of diabe-tes was done which revealed SGOT and ALT always in higher range which was always thought to be fatty liver related, but looking back patient had haemochromatosis from the begining and diabetes was secondary to iron deposition in pancreas.

following confirmation of a diagnosis of hemochromatosis, he was started on venesection. family screening was encouraged and resulted in the finding of asymptomatic diabetes associated with hemochromatosis in his brother. The blood sugars improved on in-sulin and A1C improved to 7%. The patient was found to have hy-pogonadotrophic-hypogonadism (loss of libido, erectile dysfunction and low testosterone) secondary to his haemochromatosis. He was started on six-weekly testosterone injections with good effect. He also had an MRI scan of his pituitary which was normal.

Keywords: Diabetes, Hypogonadism,Haemochromatosis

P – 08 diabetes

diaBetes and HyPOgOnadism: tHink Of HaemOCHrOmatOsis!

fAISAL HAMzA DALVI1, MAzHAR HAMJA DALVI2

1 aster meDIcal centre, DUbaI, UnIteD arab emIrates 2 Department of DIabetes anD enDocrInolgy,al noor HospItal,abU DHabI,UnIteD arab emIrates

2016


Context: Recently a few studies showing ability of a cell to differ-entiate into insulin–producing β-cells after prolonged duration of diabetes and reconstruction of new insulin–producing cells in the absence of autoimmunity in mice with completely ablated β-cells were reported. Here, we present a patient with type 1 diabetes and increased fasting and mixed-meal stimulated C-peptide levels 10 years after the diagnosis.

Case Description: A female patient with type 1 diabetes for 10 years was referred to our endocrinology clinic for hypoglycemia at-

tacks. Her fasting glucose, HbA1C, fasting and mixed-meal stimu-lated C-peptide levels were measured three years ago and recently. fasting and mixed-meal stimulated C-peptide levels were found to be increased compared to the levels of three years ago (<0.01 ng/mL vs. 0.06 ng/mL and <0.01 ng/mL vs 0.28 ng/mL respectively).

Conclusion: It is possible that β-cell function may increase in type 1 diabetes, a decade after the diagnosis as observed in our patient.

Keywords: type 1 diabetes, β-cell, mixed-meal stimulated C-pep-tide

P – 14 diabetes

inCreased fasting and stimulated C- PePtide levels after tHe diagnOsis Of tyPe 1 diaBetes

GONCA TAMERmeDenIyet UnIversIty, göztepe traInIng anD researcH HospItal, IstanbUl, tUrkey

2016


Background: Disease (GD) is characterized by deficient acid b-glu-cosidase activity and it is the most common lysosomal storage disorder. Recent articles showed that women with GD can have successful pregancies with or without replacement thrapies. Pa-tient’s informed decision to continue or not for treatment should be respected. Aside from this clinicans need keypoint to be sure for continuing or discontinuing the therapy during the pregnancy. According to our two cases we realized challenges that occurred throughout prior pregnancy can be a waypoint for the current preg-nancy. Despite the lack of supporting studies, it may be advisable to consider prior events during previous pregnancies that can illu-minate clinicians about continuing or discontinuing the treatment throughout current pregnancy. Cases: Our patients are women and they have type 1 Gaucher disease (GD).

Patient 1 was diagnosed at age 15 years due to abdominal enlarge-ment and bone pain. She had been treated with imiglucerase (30 U/kg every 2 weeks) since 23 years old. At age 23 she became preg-nant. This was her second pregnancy. Her first pregnancy was an uncontrolled pregnancy and she did not take replacement therapy, she experienced severe bleeding problems. After this uncontrolled and drug free period we started imiglucerase therapy to the pa-tient. After 1 year of replacement therapy she got pregnant again. She continued replacement therapy during her pregnancy with the same dose. There was no additional laboratory parameter change. Her clinical state was stable and there was no unexpected event

during her pregnancy. The patient delivered a male infant with elective cesarean section. The infant’s birth vital parameters were good and there was no additional health problem with the infant.

Patient 2 was 33 years old. She underwent elective splenectomy at age 26 years old. She was treated with 20-30 units/kg of imiglucer-ase every two weeks. She was diagnosed with severe thrombocyto-penia, bone pain, fatigue and splenomegaly. She became pregnant at age 32 years and with informed consent she discontinued her re-placement therapy throughout all pregnancies. This was her third pregnancy and she discontinued replacement therapy during her previous pregnancies. According to patient assertion there was no health problem during both 2 pregnancies and deliveries. A con-servative approach with close monitoring of mother and baby was done during the pregnancy. In the 38th week of pregnancy a healthy male infant was delivered with cesarean section. There was an un-eventful delivery and both mother and infant were healthy.

Conclusion: In conclusion we herein present two successful preg-nancies and breastfeeding period in female patient with Gaucher disease. We believe that prior experienced events during the pre-vious pregnancies should be a good waypoint to predict the risk of undesirable events throughout the current pregnancy and breast-feeding.

Keywords: Gaucher disease, enzyme replacement therapy, preg-nancy

P – 18 female reproductive endocrinology

is PriOr PregnanCy a key POint fOr rePlaCement tHeraPy deCisiOn fOr Current

PregnanCy in Patients WitH gauCHer disease?zEyNEL ABiDiN SAyINER, AyTEN ERAyDIN, MESUT ÖzKAyA, ERSiN AKARSU, SUzAN TABUR,

MUSTAfA ARAzDepartment of enDocrInology anD metabolIsm, gazIantep UnIversIty, gazIantep, tUrkey

2016


Introduction: Multiple Endocrine Neoplasia-1 (MEN-1) is a rare syndrome (1/30000) which has autosomal dominant genetic trans-mission but sporadical occurrence is also reported. We report herein a MEN-1 case presented with hyperinsulinemic hypoglyce-mia diagnosed both hyperparathyroidism and asymptomatic hy-perprolactinemia.

Case: A twenty-eight years old man had been suffering with fre-quent hunger symptoms for five-six years. When he was admit-ted to emergency room with complaints of perspiration and loss of consciousness, his blood glucose was found 35 mg/dl, after dex-trose infusion his glucose was improved. His blood pressure was 120/75 mmHg, heart rate was 78 /min.; his height and weight were 180 cm and 108 kg respectively, with body mass index of 33.3 kg/m2 on admission. In prolonged fasting hypoglycemia test, at 18 hours of prolonged supervised fasting test (Table 1) his blood glucose was 38 mg/dl; insulin was 10,83 uIU/mL. C-peptide was 1,51 ng/mL, urinary ketone was also negative. The MR imaging revealed two mass lesions that the first was sized 23x20 mm lobulated count-er (border) in level of pancreatic body –tail section and the second one was approximately sized 13 mm in uncinate process. Because of the patient’s history of nephrolithiasis; hypercalcemia and hy-perparathyroidism were observed (Table 2) Primary hyperparathy-roidism was considered as a part of MEN-1. Bilaterally parathyroid adenomas were observed in parathyroid ultrasonography (figure 3 and 4) and scintigraphy. There were regular hypoechoic nodular lesions suspicious of parathyroid adenomas that are 6,5*9,1*13,1 mm sized in left side and 13,6*11,3*16 mm sized in right side. Parathyroid washout results were respectively 3482 pg/dL in right lesion and 2149 pg/dL in left lession.

Our patient had both insulinoma and primary hyperparathyroidism so that we checked anterior pituitary hormones and we found that prolactin level was high (205 ng/mL). After that pituitary MRI was revealed a macroadenoma sized 14*12 mm extending to the su-prasellar cistern, isointense in T1 section and heterogenous en-hancing in T2 section. Visual field was normal. According to clin-ical, radiological and hormonal results; the diagnosis of Multiple Endocrine Neoplasia -1 (MEN-1)-Wermer syndrome was verified.

Our patients underwent total pancreatectomy for insulinomas, fol-low up parathyroid adenomectomies. Also cabergoline was admin-istered for his prolactinomas. He is referred to genetic analyses.

Discussion: MEN 1 syndrome is characterized with primary hyper-parathyroidism (90%); pancreatic neuroendocrine tumors (75%) and pituitary adenomas (35%) In our patient multiple insulinoma lesions were detected as first presentantion, in addition as a part of MEN-1 syndrome, bilateral parathyroid adenomas and prolac-tinoma were diagnosed concomitantly. According to our case, we suggest that clinical expression of the MEN-1 syndrome is variable, and its components can be diagnosed simultaneously with careful examinations.

Keywords: MEN1, insulinoma, primary hyperparathyroidism, pro-lactinoma

figure 1. PANCREAS MRI

P – 19 neuroendocrine tumors

a men1 Case Presented WitH HyPerinsulinemiC HyPOglyCemia

DiLEK KARAKAyA ARPACI1, MUSTAfA üNAL1, HANDE ORAN2, iLKER Öz3, TANER BAyRAKTAROğLU1

1 DIvIsIon of enDocrInology anD metabolIsm, Department of Internal meDIcIne, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey

2 Department of raDIology, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey 3 Department of Internal meDIcIne, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey

2016


figure 2. Pancreas MRI

figure 3. Parathyroid USG

figure 4. PARATHyROID USG

figure 5. Pituitary MRI

Table-1 Results of the prolonged fasting test

Results 0. 12. h. 18.h.

Glucose (mg/dL) (74-100) 107 50 38

Insulin (uIU/mL) (3-25) 65,76 10,91 10,83

C-peptide (ng/mL) (0.9-7.1) 9,33 2,09 1,51

Table 2.Baseline laboratorial results

Parameters Results

ParathormonH (pg/mL) (15-65) 133,9

Alkalen phosphatase (U/L)(30-120) 118

Calcium (mg/dL) (8.5-10.5) 12,7

Phosphorus (mg/dL) (2,5-4,5) 2,7

Albumin (g/dL) (3,5-5,2) 5,1

25 OH D vitamin (ng/mL.) (30-100) 10,3

24-hour urinary calcium (mg/a day) (100-300) 532

Creatinine clearance (mL/ minute) 139

Prolactin (ng/mL) 205

Prolactin levels after precipitated with PEG (ng/mL) 289

follicule Stimulating Hormone (m IU /m L) 1,74

Luteinizing Hormone (m IU /m L) 2.47

Total testosterone (ng/m L) (1,75-7,81) 2,28

Cortisol (µ IU/mL) (3.7-19.4) 19

Adrenocorticotropin (pg/mL) (10-46) 70,7

Throid Stimulating Hormone (µ IU/mL) (0.33-4.2) 1,37

Growth Hormone (ng/mL) (0.05-8.6) 0,35

2016


Introduction: Persistent hyperinsulinemic hypoglycemia is caused commonly by insulinoma or by nesidioblastosis. This disease is characterized by persistent hypoglycemia as a result of hyperse-cretion of insulin from insulinoma or β-cell hyperplasia of the pan-creas in adults representing 0.5%-5% of cases of organic hyperin-sulinemia.

We report a very rare case with persistent hyperinsulinemic hypo-glycemia due to hypersecretion of insulin from β-cell of the pan-creas.

Case: A 61-year-old woman was referred to our center because of persistent and repeated severe hypoglycemia. Her past history included the diagnosis of essential hypertension treated vith nebiv-olol 5 mg per day and recovered facial paralysis. The physical ex-amination revaled the following: height 168cm, weight 80 kg, blood pressure 120/70 mmHg, pulse 67/minutes. No other signs were detected in her system examinations. Hyperinsulinemic hypogly-caemia was diagnosed with insulin level >300 uIu/ml and C peptide level 13.50 nmol/l during 35 mg/ml fasting blood glucose. Imag-ing modalities and laboratory examination, including repeated CT, MRI scans and endosonography were negative. Subsequent testing

involved stimulation of the pancreas through cannulation of the pancreatic vascular supply by interventional radiology. Selective arterial calcium stimulation test revealed marked insulin release to calcium challenge across multiple pancreatic segments (Table 1). Insulinoma was not visualized by angioraphy during stimulaton test. On the follow up, the patient rejected the operation, and was treated with somatostatin analogs and diazoxide.

Conclusion: Once the diagnosis of hyperinsulinemic hypoglycae-mia was made, patients underwent preoperative localization by US, CT and/or MRI. One or several non-invasive radiological imaging procedures and selective arterial calcium stimulation test were performed, to referred patients with hyperinsulinemic hypoglycae-mia after a negative endosonographical localization of insulinomas. Selective arterial calcium stimulation test and endosonography of pancreas are complementary procedures and sometimes may be coupled to reach maximal diagnostic performance for localization of the tumor.

Keywords: hyperinsulinemic hypoglycemia, insulinoma,nesidio-blastosis


tHe lOCalizatiOn Of tHe OCCult insulinOma By seleCtive arterial CalCium stimulatiOn

test in a Patient WitH Persistent HyPerinsulinemiC HyPOglyCemia

MUSTAfA üNAL1, DiLEK ARPACI1, iLKER Öz2, ECE KONUK3, TANER BAyRAKTAROğLU1

1 DIvIsIon of enDocrInology anD metabolIsm, Department of Internal meDIcIne, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey

2 Department of raDIology, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey 3 Department of Internal meDIcIne, facUlty of meDIcIne, bUlent ecevIt UnIversIty, zongUlDak, tUrkey

Table 1. Selective arterial calcium stimulation test (SACT)

Time (Seconds 0. 30. 60. 90. 120. 180. 300.

Proximal splenic artery Insulin (uIU/ml) 1228 1252 1336 1257 1285 1243 98

Glucose (mg/dl) 176 174 176 177 176 177 165

Superior mesenteric artery Insulin (uIU/ml) 1380 1264 1330 1338 1349 1333 1370

Glucose (mg/dl) 178 178 174 177 172 179 180

Gastroduodenal artery Insulin (uIU/ml) 1096 1171 1210 >300 >300 297 21

Glucose (mg/dl) 173 170 170 174 169 172 170

Hepatic artery Insulin (uIU/ml) 1098 1091 1112 1274 1334 1120 1049

Glucose (mg/dl) 155 150 158 154 174 179 177

Intrahepatic artery Insulin (uIU/ml) 1241 1302 1288 1274 1334 1287 1260

Glucose (mg/dl) 175 177 178 177 174 179 177

2016


Introduction: Though medullary thyroid carcinoma (MTC) accounts for 5 to 10 % of all thyroid cancers. the prognosis is worse than differiantiated thyroid carcinoma. It was reported as 10-year sur-vival of 40-90 %. The hereditary form is 25 % and sporadic form is 75%. The variable clinical course of patients was reported because of a relatively slow tumour growth but early lymphatic metastatic spread. We are presenting a patient with asymptomatic but bio-chemically active and clinically persistent MTC.

Clinical Case: Diagnostic evaluation and treatment

A 40-year old man presenting with a palpable thyroid nodule. Ul-trasonography showed calcific 18 mm nodule in the left lobe and calcific 30 mm lymph node in the left juguler. Cytologic examina-tion revealed MTC both in thyroid and lymph node in tle left side. Total thyroidectomy and lymph node dissection showed multifocal (2.5 cm in the left lobe and 0.6 cm in the right lobe) with lympho-vascular and locoregional invasion of MTC but also positive me-diastinal lymph node metastasis. Postoperative serum calcitonin was 352 pg/m L and whole body scanning withTc-99M V-DMSA re-vealed pathologic accumulation on the left side of neck. After three months he was reoperated as sternotomy with bilateral lymph node dissection and serum calcitonin was decreased to 83 pg/mL.

He was asymptomatic and urinary catecholamines were normal and showed no hepatic metastasis with computed tomography.

Follow-Up: After seven years he was referred for control. Serum calcitonin was 104 pg/mL and US showed supraclavicular sol-id masses (22 and 19 mm) with submandibular pathologic lymph node (11 mm) in the left side. Positron emission tomography (PET) using 18f-fDG confirmed hypermetabolic nodular lesion. Cyoto-logic examination from lymph node was non diagnostic.

After six months serum calcitonin was 108 pg/mL. The volumet-ric magnetic resonance imaging showed bilateral multiple cervical lymph nodes (the largest was 13 mm in the right side) and anterior prevertebral soft tissue lesion in the right side at the level of 1 st intercostal space (17 mm) and two lymph nodes in the right para-tracheal region at the level of upper mediastinum. The incisional biopsy showed connective tissue. PET using(18f-DOPA) revealed pathologic accumulation.

We suggested to the patient to test for Rearranged during transfec-tion (RET) gene but he did not want to do.

Conclusion: MTC shows slow tumour growth with early lymphat-ic metastasis and postoperative increased serum calcitonin levels suggests persisting tumour and different imaging techniques re-veal clinically persistent disease. Age at diagnosis, tumour stage and genetic analysis are important factors in determining progno-sis. We suggested the third operation to our patient considering a prolonged indolent course of disease even with metastasis.

Keywords: medullary, thyroid,carcinoma, persistent, disease


a Case rePOrt: BiOCHemiCally and CliniCally Persistent medullary tHyrOid CarCinOma

fOr eigHt yearsşERifE NUR BOySAN1, GüziDE ÖzDiL2, EMEL GüNAy3, ÖNDER PEKER4, METE DüREN5,

MUSTAfA SAiT GÖNEN6

1 IstanbUl bIlIm UnIversIty, enDocrInology, IstanbUl, tUrkey 2 IstanbUl bIlIm UnIversIty, raDIology, IstanbUl, tUrkey

3 IstanbUl, bIlIm UnIversIty, nUclear meDIcIne, IstanbUl, tUrkey 4 amerIcan HospItal, IstanbUl, tUrkey

5 IstanbUl UnIversIty,cerraHpasa meDIcal facUlty, IstanbUl, tUrkey 6 IstanbUl bIlIm UnIversIty, enDocrInology, IstanbUl, tUrkey

2016


Objective: Intrasellar aneurysms originated from the cavernous carotid are rare. This type of aneurysms can simulate pititary ad-enomas. It is crucial to confirm an aneurysms suspicion with con-ventional angiography before surgery for a pituitary mass. There is not always a relationship between headaches and pituitary ad-enomas. Therefore, the treatment of pituitary tumors does not al-ways result in resolution of headaches. Besides headache is not a surgery indication for surgery. But if headache pattern is severe or unstable detailed evalution of pituitary region should be done.

Case: A 45-year-old female patient was admitted to neurology out-patient clinic complaining of headache and fatigue. Neurologist performed cranial magnetic resonance imaging. They found out pi-tuitary adenoma and they referred the patient to our endocrinology outpatient clinic. Pituitary adenoma was in the supraselar region and it was located near internal carotid artery. It was 13x14 mm in diameter. We evaluated the patients whether the tumor is hormone secreting or not. We did not find any sign of hormone secretion in physical examination and biochemical tests. Patient has no other symptom except from the headache. It was thought that the patient had non secreting pituitary macro adenoma. The patient’s head-ache was getting worse during the evaluation. We hospitalized the patient and we reevaluated the MRI of the patient. There was round shape adenoma but the border of the adenoma was very regular and it was like a coin shape. Moreover, it had low signal on MRI. (figure 1) We decided to perform 3 dimensional computer tomog-raphy of brain. (figure 2) It showed us saccular aneurysm which is originated from ophthalmic branch of internal carotid artery and it was located in the supraselar region. Then we performed cerebral angiography and confirmed the aneurysm.

Conclusion: Headache pattern be a clue for differential diagnosis in patients with pituitary mass. As shown in our case there can be different causes other than the adenomas. Patients who have se-vere headache or change in the headache pattern with pituitary ad-enoma make us think that there can be another reason aside from adenoma such as aneurysms.

Keywords: Pituitary mass, headache, aneurysm

figure 1. Pituitary adenoma with low signal on MRI

figure 2. 3D Computed tomography angiography of the aneurysm

P – 22 Pituitary

may HeadaCHe Pattern Be a Clue fOr differential diagnOsis in Patients WitH

Pituitary mass?zEyNEL ABiDiN SAyINER1, AyTEN ERAyDIN1, iBRAHiM ERKUTLU2, ERSiN AKARSU1, MUSTAfA ARAz1

1 Department of enDocrInology anD metabolIsm, gazIantep UnIversIty, gazIantep, tUrkey 2 Department of neUrosUrgery, gazIantep UnIversIty, gazIantep, tUrkey

2016


Introduction: McCune Albright Syndome (MAS) includes the clas-sic triad of poly/monostotic fibrous dysplasia (fD), cafe-au-lait spots and hyperfunctioning endocrinopathies (hyperthyroidism, acromegaly and cushing’s syndrome). This syndrome is character-ized by autonomous hyperfunction of variety of cells that respond to extracellular signals through activation of the hormone sensitive adenylate cyclase system, which catalyzes the production of cAMP. Acromegaly affects around 20% of MAS patients. We report here a patient with MAS and acromegaly.

Case: A 53-year old women presented with sweating, palpitation and neck swelling at 38 years old in 2001. She was diagnosed with bone fibrous dysplasia of the skull, which was polyostotic at 4 years old. She had short stature and precocious puberty. The patient was hyperthyroid and uptake was increased at the right side of the thy-roid. Thyroid ultrasonography revealed many nodules, the greatest being 38x30 mm in the right lobe. fine needle aspiration cytolo-gy was benign. Radioactive iodine (RAI) therapy was given in 2002. After RAI, the patient was hypothyroid and levothyroxine therapy was begun. Hypertension, fatty liver disease and diabetes mellitus developed in 2012. The craniofacial fibrous dysplasia lesions grew and serum growth hormone level was 7.2 ng/mL and IGf-1 was 979 ng/L (94-252). Serum growth hormone level was not suppressed by oral glucose tolerance test.

Octreotide 20 mg was begun and cabergoline was added. The med-ical therapy was ineffective even though the cabergoline (DA) dose was increased up to 2 mg per week and octreotide (SA) dose was up to 40 mg per month for 4 years of treatment. Surgical removal of the pituitary adenoma was technically difficult, due to the thickness of the cranial dysplasia at the skull base and the risk of hemor-rhage given the high vascularity of fibrous dysplasia.

Conclusion: The diagnosis of acromegaly in a patient with MAS may be delayed due to craniofacial fibrous dysplasia, masking the dysmorphic craniofacial effects of acromegaly. Pituitary surgery may be difficult or impossible in patients with MAS. Total hypoph-ysectomy is generally required due to diffuse involvement of the

pituitary. for these reasons, medical treatment is preferred in pa-tients with MAS-associated acromegaly. SAs decrease GH/IGf-1 levels in most patients but do not achieve normal levels generally. GH receptor antagonist pegvisomant is often effective. Malignant transformation of fibrous dysplasia is possible due to irradiation of bone lesions or GH/IGf-1 excess itself. Pituitary irradiation should be avoided and diagnostic irradiation should be kept to a minimum.

Keywords: McCune Albright Syndome, Hyperthyroidism, Acro-megaly, Cushing

figure 1. Magnetic resonance imaging in coronal view of microad-enoma (9 mm) in the left side of the pituitary gland surrounded by cranial fibrous dysplasia with MAS and acromegaly

figure 1. 2015 Hipofiz - T1-Coronal

P – 23 Pituitary

aCrOmegaly and mCCune-alBrigHt syndrOme: Case rePOrt

DiLEK yAzICI, fARUK ALAGÖL, HAVVA SEzERDeparment of Internal meDIcIne, koc UnIversIty scHool of meDIcIne, IstanbUl, tUkey

2016


A 30 years old woman was using propylthiouracil for 3 years due to hyperthyroidism. Although increased thyroid function was found, the gland was normal in ultrasonography (USG). Abdominal USG detected a cystic pelvic mass in left ovary. Magnetic resonance im-aging showed 60 mm × 45 mm cystic mass in left ovary. Radio-iodine uptake (10 μCi) and thyroid scintigraphy with 185 MBq (5 mCi) Tc99m were also normal. A whole body scan performed 48 h after oral ingestion of 29.6 MBq (0.8 mCi) iodine131 (I131) cap-sule, revealed a round structure located to the left lower abdomen. Iodine uptake was detected in this cyst that was compatible with functional thyroid tissue demonstrated by single photon emission computerized tomography/computerized tomography (SPECT/CT) [figure 1]. Left salpingooophorectomy was done with frozen sec-tion. Histopathology confirmed mature cystic teratoma that was containing thyroid follicles. The euthyroid state was achieved. Stru-ma ovarii is a rare and usually a benign ovarian teratoma, which consist of thyroid tissue in the ovarian mass. These tumors are associated with hyperthyroid disease in 5–20%. Clinical features may resemble ovarian malignancy and rarely can be metastatic. Typically presents as a multicystic mass with lobulated surface and thickened septic or cyst walls with some solid components. High attenuation areas and calcifications in the solid components are common findings on CT images. Scintigraphy with Tc99m pertech-netate, I123, and I131 were used for diagnosing a hyperfunction-ing struma ovarii on the basis of higher uptake of the radionuclide with planar images. If the clinicians or nuclear medicine physician do not consider the extrathyroidal tissue as a result of hyperthy-roidism, patient’s diagnosis cannot be made by the other nuclear medicine test such as I131 uptake and thyroid scintigraphy. So, the extrathyroidal cause of hyperthyroidism can lead to the dilemma in nuclear medicine. In our patient, an I131thyroid uptake was in normal ranges, and a Tcpertechnetate scan of the thyroid showed normal distribution. I131 whole body scan identified the cause of hyperthyroidism. further, SPECT/CT localized the thyroid tissue in large pelvic mass accurately.

Keywords: Hyperthyroidism, singlephoton emission computerized tomography/computerized tomography, struma ovarii

figure 1. Whole-body scan was performed 48 h after oral ingestion of 29.6 MBq (0.8 mCi) I-131 sodium iodide capsule revealed a focal uptake.(a) Planar image shows focal iodine uptake located to the left lower abdomen. In single-photon emission computerized tomog-raphy/computerized tomography images this focal uptake was corresponding to the solid components of the large cyst which is containing calcification (arrow). (b) Low dose unenhanced comput-erized tomography without intravenous contrast images. (c) Axial fusion single-photon emission computerized tomography/comput-erized tomography images. (d) Coronal fusion single-photon emis-sion computerized tomography/computerized tomography images

P – 24 thyroid

aCCurately lOCalizing tHe tHyrOid tissue in mature CystiC teratOma Of Ovary By single

PHOtOn emissiOn COmPuterized tOmOgraPHy/ COmPuterized tOmOgraPHy

RIfKI üçLER1, yUSUf DEMiR2, iSMET ALKIş3, GüLAy BULUT4

1 Department of enDocrInology, yüzüncü yIl UnIversIty, van, tUrkey 2 Department of nUclear meDIcIne,yüzüncü yIl UnIversIty, van, tUrkey

3 Department of obstetrIcs anD gynecology, yüzüncü yIl UnIversIty, van, tUrkey 4 Department of patHology, yüzüncü yIl UnIversIty, van, tUrkey

2016


Introduction: Although methimazole (MMI) and propylthioura-cil (PTU) have long been used to treat hyperthyroidism caused by Graves’ disease (GD), there is a tendency to use MMI depending on its lower hepatotoxicity and better treatment results. However PTU would be addressed when MMI is unavailable, counting on its comparable effect to MMI.

Case1: An 18 year-old girl was referred to our clinic with palpi-tations, weight loss and irritability. Her blood test results showed Thyrotropin (TSH):0,00 µIU/ml, free T4 (fT4):3,54 (0,89-1,79) ng/dl, free T3 (fT3):17,57 (2,3-4,2) pg/ml, anti-thyroid peroxidase:426 IU/ml while thyroid ultrasonography(USG) revealed mild heterogeneity with no nodule and thyroid 99m Technetium-pertecnatate scintig-raphy showed diffuse increased uptake. GD was diagnosed and MMI 30 mg/day was started. After MMI was realized to be out of stock, PTU 300 mg/day was prescribed. At second visit after one month she had TSH: 0,00 µIU/ml, fT4:2,74 ng/dl, fT3:12,24 pg/ml which heralded increasing the dosage to 450 mg/dl. Next time her blood test retrieved much better results with fT4:1,87 ng/dl, fT3:6,74 pg/ml. Since she was willing to have a tonsillectomy operation in re-cent time we used the same dosage. However, her hormone levels was not normal at next visit that we increased PTU to 600mg/day. One month later she had TSH: 0,00 µIU/ml, fT4:1.76 ng/dl, fT3: 7.12 pg/ml. MMI availability after that time allowed us to start MMI at 60 mg dosage. On 15 days of MMI fT3 and fT4 decreased to normal. Waiting TSH to get lower, 40 mg MMI was used and normal TSH

with fT4:0.377 ng/dl and fT3:1.83 pg/ml was provided. This essen-tialized to lower the dosage further. On 10 mg MMI, euthyroidism was managed and the patient underwent operation.

Case 2: A 50-year old man arrived at clinic with fatique and palpi-tations. His blood test results were: TSH:0.00 µIU/ml, fT4:3.6 ng/dl, fT3:8.7 pg/ml. Thyroid USG and scintigraphy showed toxic diffuse multinodular goitre. MMI 30 mg/day was started. Since unavail-ability of the drug PTU 300 mg/day was chosen. After one week fT4:2.55 ng/dl, fT3:8.37 pg/ml were found. Under one month treat-ment results were:TSH:0.00 µIU/ml, fT4: 2.24 ng/dl, fT3: 7.95 pg/ml. At same PTU dosage we continued with no alteration in hor-mone level and we increased the dosage to 450 mg/day. At next visit the normalization of hormones could not be managed when MMI rendered available. MMI 30 mg/d was used then. This yielded TSH:0.30 µIU/ml, fT4:1.1 ng/dl, fT3:3.8 pg/ml at only one month of therapy.

Conclusion: MMI should be used in every patient who chooses an-tithyroid drug therapy for GD except some occasions or when MMI is unavailable. PTU is preferred then. However, PTU would not be beneficial usually because of nonadherence of patient. PTU dis-placement with MMI would develop quicker and higher response, probably because of more compliance of patient. Possible role of pharmacodynamics of PTU already used might also be considered.

Keywords: Propylthiouracil, Response, Graves’

P – 25 thyroid

PrOPyltHiOuraCil seemingly lOWer resPOnsive tWO graves’ Patients

iffET DAğDELEN DURANDepartment of enDocrInology anD metabolIc DIseases, kIrIkkale yüksek İHtIsas HospItal, kIrIkkale,tUrkey

2016


aAHSEN, HiLAL 48AKARSU, ERSiN 55, 60, 65AKGüN, AyşEGüL 16AKINCI, BARIş 14, 18AKSAKAL, NiHAT 47AKyILDIz, MAHiR 15ALAGÖL, fARUK 66ALKIş, iSMET 67ALTUNBAş, HASAN ALi 9, 21, 35ALTUNTAş, yüKSEL 40ARAL, fERiHAN 47ARAL, yALçIN 29ARAz, MUSTAfA 55, 60, 65ARPACI, DiLEK KARAKAyA 61, 63AyTüRK, SEMRA 45

BBAHçECiOğLU, ADiLE BEGüM 50BAKINER, OKAN 36, 38BALCI, MUSTAfA KEMAL 9, 21,

35BAşER, HüSNiyE 48BAşPINAR, BATUHAN 25BAyKAL, GÖKHAN 51BAyRAKTAR, MiyASE 20, 54BAyRAKTAROğLU, TANER 61, 63BERKER, DiLEK 17, 30BEşTEPE, NAGiHAN 32BiLEN, CENK yüCEL 43BiLGiNER, CüNEyT 13BOySAN, şERifE NUR 64BOzKIRLI, EMRE 36, 38BOzOğLAN, HüMEyRA 9, 21, 35BULUT, GüLAy 67

CCANSU, GüVEN BARIş 44CERiT, ETHEM TURGAy 23CULHA, CAViT 29

ÇçAKIR, BEKiR 13, 32, 48çALIşKAN yILDIRIM, EDA 20çAVDAR, üMiT 14çETiN, zEyNEP 17, 30

çOLAK BOzKURT, NUJEN 29çUHACI, NESLiHAN 13, 48

dDAğDELEN DURAN, iffET 24,

57, 68DAğDELEN, SELçUK 43, 46DALMATOVA, ANNA 37DALVi, fAiSAL HAMzA 58DALVi, MAzHAR HAMJA 58DEMiR, TEVfiK 14DEMiR, yUSUf 67DiRi, HALiT 18DOğAN, ÖzLEM 9, 21, 35DOğRUEL, HAKAN 9DOğRUL, AHMET BüLENT 46DüREN, METE 64

eEKşi HAyDARDEDEOğLU, fiLiz 36, 38ELBOğA, UMUT 55ERAyDIN, AyTEN 55, 60, 65ERBAş, TOMRiS 43ERDOğAN, CAN EMRE 27ERKUTLU, iBRAHiM 65ERSOy, CANAN 11ERSOy, REyHAN 13, 32, 48ERSÖz GüLçELiK, NEşE 24, 29ERTAN, yEşiM 10ERTÖRER, MELEK EDA 36, 38ERTüRK, ERDiNç 11EyüPOğLU, DAMLA 26

ffAKI, SEVGüL 13fIRAT, SEVDE NUR 29

gGÖNEN, MUSTAfA SAiT 64GRiNEVA, ELENA 37GüLER, SERDAR 17, 30GüL, NURDAN 22GüNAy, EMEL 64GüRER, iNANç ELif 35GüRLEK, ALPER 8, 50GüVEN, DENiz CAN 8

INDEXH

HACIşAHiNOğULLARI, HüLyA 22HAN, üNSAL 13HELVACI, NAfiyE 8, 20, 51

kKALAyOğLU BEşIşIK, SEVGi 22KARACA, ANARA 29KARAGüLLE, MUSTAfA 44KARAKOç, DERyA 52KARCI, ALPER çAğRI 17, 30KARDAş, RIzA CAN 46KARDES, ÖzGüR 36KARONOVA, TATiANA 37KARTAL, iLKAy 19KAV, TAyLAN 25KAyASELçUK, fAziLET 36KILIçARSLAN, AyDAN 32KILIç, MEHMET 32, 48KIyICI, SiNEM 45KLyAUS, NATALiA 37KOCA, MELTEM 52KOç, GÖNüL 29KONUK, ECE 63KORKMAz, HAKAN 13KUBAT üzüM, AyşE 22, 47KUzU, iDRiS 40

mMAKAy, ÖzER 16MALAy, ÖzER 10MAMMADOVA, AfRUz 54MESTANzADE, METBAN 22METiN, MELiKE RUşEN 32

nNASIROğLU iMGA, NARiN 17, 30NiKA, CHRiSTiNA 34

OOğUz, SEDA 8, 25, 43, 46, 52, 56OMMA, TüLAy 29ORAN, HANDE 61

2016


ÖÖğMEN, BERNA 13ÖğüT, TAHiR SAyGIN 21, 35ÖNDER, SEMEN 47ÖzDEMiR, DiDEM 32ÖzDEMiR KUTBAy, NiLüfER 15,

16, 18, 27ÖzDEMiR, MURAT 10ÖzDiL, GüziDE 64ÖzENMiş, TAHSiN 29ÖzGEN, GÖKHAN 10, 15, 16ÖzIşIK, HATiCE 10, 15, 16, 18, 27Öz, iLKER 61, 63ÖzKAyA, MESUT 55, 60

PPAPATHEODOROU, ATHANASiOS 34PASCHOU, STAVROULA A. 34PEKER, ÖNDER 64PEyNiRCi, HANDE 11POLAT, şEfiKA BURçAK 32POyRAz, NiüUfER 48

qQASiM, BAyAR AHMED 31, 49

sSALiM, OzAN 21SAMARAS, VASSiLiS 34SARAç SiVRiKOz, TUğBA 47SARI, RAMAzAN 9, 21, 35SAyDAM, BAşAK ÖzGEN 14SAyGILI, füSUN 18, 27SAyINER, zEyNEL ABiDiN 55,

60, 65SEzER, HAVVA 66SOyLUK SELçUKBiRiCiK, ÖzLEM 22SÖKMENSüER, CENK 43, 46SüLEyMANLAR, GüLTEKiN 35

ŞşARER yüREKLi, BANU 10, 15, 16, 18,

27şiMşEK BAğIR, GüLAy 36, 38şişMAN, PINAR 11

tTABUR, SUzAN 55, 60TAMER, GONCA 59TANAKOL, REfiK 22TANRIKULU, SEHER 47TAşKIRAN, BENGüR 11, 44TATLIPINAR, ARzU 19TAzEGüL, GÖKHAN 21, 35TEKiN, SAKiN 47

TOPALOğLU, OyA 13, 48TOPçUOğLU, ELif 29TSiBERKiN, ALEXANDR 37TUNCAy, MEHMET 23TziORAS, KONSTANTiNOS 34

uUyGUN, BURçiN 45

ÜüçLER, RIfKI 67üNAL, MUSTAfA 61, 63üNLüTüRK, UğUR 20, 26, 52

vVAHABOV, JAVANSHiR 56VETEM KARAGüLLE, iNCi 44VRyONiDOU, ANDROMACHi 34

yyALçIN, MEHMET MUHiTTiN 23yAşAR, zEKi 18yAzICI, DiLEK 66yILDIRIM, MELTEM 48yILDIRIM şiMşiR, ILGIN 10, 27yILDIz, BüLENT OKAN 25, 51, 56yILMAz, NUSRET 9, 21, 35

zzUHUR, SAyiD 40

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Bridging the World of Endocrinology

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2016 - Endo Bridgeendobridge.org/arapca/cases_2016.pdf · 2016 6 20 - 23 October, 2016 Antalya - Turkey DATE TIME HALL SESSION TITLE 22.10 11.20-12.50 A Diabetes O-21 / The investigation