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Malignant Thyroid Disease Ong, Edilisa Onilla, John Christopher 3 Medicine C Slide 2 39 y/o Female CC: Anterior Neck Mass Ong, Vicar Slide 3 10 years PTC Slowly growing nodular Anterior Neck Mass No other accompanying symptoms 2 years PTC Rapid Increase in the size of the mass 6 months PTC hoarseness Difficulty in swallowing HISTORY OF PRESENT ILLNESS: Ong, Vicar Slide 4 REVIEW OF SYSTEMS: NO Fever Weight Loss TremorsChest Pain Easy Fatigability Abdominal Pain Ong, Vicar Slide 5 PAST MEDICAL HISTORY Unremarkable FAMILY HISTORY Unremarkable Ong, Vicar Slide 6 PHYSICAL EXAM PR = 100/min RR = 20/min T = 37 0 C No Exopthalmos Neck: 25X20cm multinodular, firm, right anterolateral neck mass which moves with deglutination there is a mass 5X3cm hard nodule within the big mass Palpable cervical adenopathies posterior to the SCM Ong, Vicar Slide 7 Salient features 39 y/o Female Lives in Bicol Ten year history of a slow growing nodular anterior neck mass 2 years prior to consult- rapid increase in size of mass; 25x20 firm, right, anterolateral, moves with deglutition. 5x3cm hard nodule within the big mass 6mos PTC- hoarseness and difficulty swallowing Palpable cervical adenopathies posterior to the sternocleidomastoid Ong, Keno Slide 8 Clinical Impression Papillary Carcinoma accounts for 80% of all thyroid malignancies occurs more often in women, with a 2:1 female:male ratio mean age at presentation is 30 to 40 years euthyroid and present with a slow-growing painless mass in the neck Dysphagia, dyspnea, and dysphonia are usually associated with locally advanced invasive disease Lymph node metastases are common Ong, Keno Slide 9 Differential diagnosis Goiter may be diffuse, uninodular, or multinodular asymptomatic, although patients often complain of a pressure sensation in the neck, particularly with motion compressive symptoms, such as dyspnea and dysphagia Dysphonia from recurrent laryngeal nerve injury is rare, except when malignancy is present soft, diffusely enlarged gland (simple goiter) or nodules of various size and consistency in case of a multinodular goiter Ong, Keno Slide 10 Follicular Carcinoma 10% of thyroid cancers Common in iodine deficient areas Women have a higher incidence of follicular cancer, with a female:male ratio of 3:1, and a mean age at presentation of 50 years solitary thyroid nodules, occasionally with a history of rapid size increase, and long-standing goiter cervical lymphadenopathy is uncommon at initial presentation approximately 5% Ong, Keno Slide 11 Hurthle Cell 3% of all thyroid malignancies more often multifocal and bilateral (approximately 30%) usually do not take up RAI (approximately 5%), more likely to metastasize to local nodes (25%) and distant sites associated with a higher mortality rate (approximately 20% at 10 years) Slide 12 Medullary Carcinoma 5% of thyroid malignancies and arise from the parafollicular or C cells of the thyroid present with a neck mass that may be associated with palpable cervical lymphadenopathy (15 to 20%) Local pain or aching is more common in patients with these tumors, and local invasion may produce symptoms of dysphagia, dyspnea, or dysphonia female:male ratio is 1.5:1 Most patients present between 50 and 60 years of age frequently develop diarrhea, which may result from increased intestinal motility and impaired intestinal water and electrolyte flux Ong, Keno Slide 13 Anaplastic Carcinoma Women are more commonly affected majority of tumors present in the seventh and eighth decades of life long-standing neck mass, which rapidly enlarges and may be painful Associated symptoms, such as dysphonia, dysphagia, and dyspnea, are common Lymph nodes usually are palpable at presentation Ong, Keno Slide 14 2. What work-ups are needed if any? Fine needle aspiration biopsy (FNAB) needle is placed into the nodule several times and cells are aspirated into a syringe Viewed by pathologist Complete neck ultrasound To evaluate lymph node metastasis and the contralateral lobe Ong, Nicodemus Slide 15 Slide 16 Slide 17 3. INDICATIONS FOR THYROIDECTOMY 1. As therapy for some individuals with thyrotoxicosis, both those with Graves disease and others with hot nodules 2. To establish a definitive diagnosis of a mass within the thyroid gland, especially when cytologic analysis after fine-needle aspiration (FNA) is either nondiagnostic or equivocal 3. To treat benign and malignant thyroid tumors 4. To alleviate pressure symptoms or respiratory difficulties associated with a benign or malignant process 5. To remove an unsightly goiter. 6. To remove large substernal goiters, especially when they cause respiratory difficulties Chapter 21. Surgery of the Thyroid Gland, Edwin L. Kaplan, MD et. al., Revised 20 Jul 2010, retrieved from http://www.thyroidmanager.org/, last November 27, 2011http://www.thyroidmanager.org/ Onilla, Arianne Slide 18 Standard Treatment of Most Papillary Carcinomas Total Thyroidectomy Near Total Thyroidectomy Chapter 21. Surgery of the Thyroid Gland, Edwin L. Kaplan, MD et. al., Revised 20 Jul 2010, retrieved from http://www.thyroidmanager.org/, last November 27, 2011http://www.thyroidmanager.org/ Onilla, Arianne Slide 19 MRND Chapter 21. Surgery of the Thyroid Gland, Edwin L. Kaplan, MD et. al., Revised 20 Jul 2010, retrieved from http://www.thyroidmanager.org/, last November 27, 2011http://www.thyroidmanager.org/ Onilla, Arianne Slide 20 Radioiodine Therapy Radioiodine therapy with I 131 has been commonly used in order to ablate any remaining normal thyroid remnant that is present in the thyroid bed after near- total or total thyroidectomy or to treat local or distant metastatic thyroid cancer. Chapter 21. Surgery of the Thyroid Gland, Edwin L. Kaplan, MD et. al., Revised 20 Jul 2010, retrieved from http://www.thyroidmanager.org/, last November 27, 2011http://www.thyroidmanager.org/ Onilla, Arianne Slide 21 4. How would you manage the patient? Immediate postop? In the next 4-6 weeks? Long term plans? Ong, Edilisa Slide 22 Postoperative Management of Differentiated Thyroid Cancer I. Radioiodine Therapy Reduces recurrence and provides a small improvement in survival, even in low-risk patients More sensitive screening tool than CXR or CT scan for detecting metastasis Less sensitive than thyroglobulin (Tg) for detecting metastatic disease (except H rtle cell tumors) Metastatic diff. thyroid CA can be detected and treated by 131 I in about 75% of patients Treats>70% of lung micrometastases at are detected by RAI scan in the presence of a normal CXR Success rates drop to Postoperative Management of Differentiated Thyroid Cancer Cont. Radioiodine Therapy Discontinue T4 therapy for approx. 6 weeks before scanning with 131 I. T3 should be given to decrease the period of hypothyroidism. T3 discontinued for 2 weeks to allow TSH levels to rise before treatment (>30mU/L) Low iodine diet Screening dose: 1-3 mCi of 123 I; mx uptake 24 hours later Should be Follow-Up of Patients with Differentiated Thyroid Cancer I.Thyroglobulin (Tg) Measurement Tg levels after total thyroidectomy should be: < 2ng/mL (if Px is taking T4)2ng/mL is highly suggestive of metastatic disease or persistent normal thyroid tissue, esp. if it increases when TSH levels increase when hypothyroid during preparation for RAI scanning or after recombinant TSH Schwartzs Principle of Surgery, 9 th edition Slide 29 95% of patients with persistent or recurrent follicular cell carcinoma will have Tg levels > 2ng/mL Tg and anti-Tg antibody levels should be measured initially at 6-month intervals and then annualy if Px is clinically disease free Schwartzs Principle of Surgery, 9 th edition Harrisons Principles of Internal Medicine, 17 th edition Ong, Edilisa Slide 30 Follow-Up of Patients with Differentiated Thyroid Cancer II. Imaging For low risk patients (-) TSH-stimulated Tg and cervical ultrasound Dont require routine Dx whole body RAI scans For high-intermediate risk patients Whole body scans 6-12 months after remnant ablation may be of value Cervical ultrasound recommended to evaluate thyroid bed and central & lateral cervical nodal compartments at 6-12 months postthyroidectomy and then annualy for at least 3-5 years If RAI and ultrasound scans are (-) but Tg remains elevated FDG PET scan may help localize the disease Schwartzs Principle of Surgery, 9 th edition Ong, Edilisa Slide 31 Survival Rate A. Papillary cancer, cohort of 1851 patients. I, 1107 (60%); II, 408 (22%); III, 312 (17%); IV, 24 (1%); n = 1185. B. Follicular cancer, cohort of 153 patients. I, 42 (27%); II, 82 (54%); III, 6 (4%); IV, 23 (15%); n = 153. Harrisons Principles of Internal Medicine, 17 th edition Ong, Edilisa Slide 32 5. What are the possible complications of treatment? Ong, Edilisa Slide 33 Complications of Thyroid Surgery Minor Postoperative surgical site seromas Poor scar formation Major Bleeding Injury to the Recurrent Laryngeal Nerve Injury to the Superior Laryngeal Nerve Hypoparathyroidism Thyrotoxic Storm Infection Hypothyroidism Slide 34 Major Complications - Bleeding Intraoperative bleeding stains the tissues and obscures important structures Postoperative bleeding unrecognized or rapidly expanding hematoma can cause airway compromise and asphyxiation Presentation neck swelling, neck pain, and/or signs and symptoms of airway obstruction (eg, dyspnea, stridor, hypoxia) Evaluation Physical examination Imaging studies (CT scanning and ultrasonography) Prevention Sound surgical technique Treatment If a neck hematoma is compromising the patient's airway, open the surgical incision at the bedside to release the collection of blood, and immediately transfer the patient to the operating room. Slide 35 Major complications Injury to the Recurrent Laryngeal Nerve Mechanisms of injury to the RLN include complete or partial transection, traction, contusion, crush, burn, misplaced ligature, and compromised blood supply True vocal-fold paresis or paralysis Presentation Patients with unilateral vocal fold paralysis present with postoperative hoarseness or breathiness Patients with bilateral vocal-fold paralysis may present with biphasic stridor, respiratory distress, or both Evaluation Indirect and fiberoptic laryngoscopy Prevention Deliberate identification of the RLN minimizes the risk of injury Slide 36 Treatment Two surgical treatment options are available for patients with unilateral vocal-fold paralysis: medialization and reinnervation. Medialization is most commonly performed Medialization of the impaired vocal fold improves contact with the contralateral mobile fold Reinnervation - primary neurorrhaphy using phrenic nerve, ansa cervicalis, and preganglionic sympathetic neurons In bilateral vocal-cord paralysis, initial treatment involves obtaining an adequate airway Emergency tracheotomy may be required Cordotomy and arytenoidectomy are the most common procedures Slide 37 Major complications Injury to the SuperiorLaryngeal Nerve The external branch provides motor function to the cricothyroid muscle involved in elongation of the vocal folds Trauma to the nerve results in an inability to lengthen a vocal fold and, thus, an inability to create a high-pitched sound The external branch of the SLN is probably the nerve most commonly injured in thyroid surgery (0-25%) Presentation mild hoarseness or decreased vocal stamina, loss of the upper register Evaluation Laryngeal EMG Prevention Deliberate identification of the SLN minimizes the risk of injury Treatment Speech Therapy Slide 38 Major complications: Hypoparathyroidism Inadequate production of PTH leads to hypocalcemia Hypoparathyroidism, and the resulting hypocalcemia, may be permanent or transient The rate of permanent hypoparathyroidism is 0.4-13.8%. The condition may be due to direct trauma to the parathyroid glands, devascularization of the glands, or removal of the glands during surgery Presentation Initially asymptomatic Symptoms and signs of hypocalcemia include circumoral paresthesias, mental status changes, tetany, carpopedal spasm, laryngospasm, seizures, QT prolongation on ECG, and cardiac arrest Prevention The best way to preserve parathyroid gland function is to identify the glands and to maintain their blood supply Treatment Patients who have symptomatic hypocalcemia in the early postoperative period or whose calcium levels continue to fall rapidly require treatment In symptomatic patients, replace calcium with intravenous calcium gluconate Slide 39 May result from manipulation of the thyroid gland during surgery in the patients with hyperthyroidism Presentation and evaluation Signs of thyrotoxic storm in the anesthetized patient include tachycardia and hyperthermia in the awake patient - nausea, tremor, and altered mental status, cardiac arrhythmias Intraoperative management Stop the procedure. Intravenous beta-blockers, PTU, sodium iodine, and steroids are administered to control sympathetic activity, release of thyroid hormone, hyperthermia Postoperative management Removal of the thyroid gland does not immediately relieve thyrotoxicosis because the half-life of circulating T4 is 7-8 days As thyroid hormone levels decrease and as symptoms resolve, medications should be gradually weaned over the weeks after surgery Major complications: Thyrotoxic storm Slide 40 Presentation Postthyroidectomy infection may manifest as superficial cellulitis or as an abscess Evaluation Gram staining and culturing to direct the choice of antibiotics CT imaging - deep neck abscess Prevention sterile surgical technique Treatment Antibiotics that provide good coverage against gram-positive organisms (eg, against staphylococci and streptococci) If patients have deep neck abscesses, begin with broad- spectrum antibiotics Drain abscesses, and direct antibiotic coverage according to culture findings Major complications: Infection Slide 41 Untreated hypothyroidism causes symptoms such as cold intolerance, fatigue, constipation, muscle cramping, and weight gain Evaluation Measurement of thyrotropin (thyroid-stimulating hormone [TSH]) levels Prevention Hypothyroidism is an expected sequela of total thyroidectomy Treatment For hypothyroid patients, start levothyroxine (about 1.7 mcg/kg/d) Check their thyrotropin level in approximately 4-6 weeks, and adjust the dosage appropriately Major complications: Hypothyroidism Slide 42 Table 38-7 Complications of Radioactive Iodine Therapy ( 131 I) and Doses at Which They Are Observed AcuteLong-Term Neck pain, swelling, and tendernessHematologic Thyroiditis (if remnant present) Bone marrow suppression (>500 mCi) Sialadenitis (50450 mCi), taste dysfunction Leukemia ( >1000 mCi) Hemorrhage (brain metastases)Fertility Cerebral edema (brain metastases, 200 mCi) Ovarian/testicular damage, infertility Vocal cord paralysisIncreased spontaneous abortion rate Nausea and vomiting (50450 mCi) Bone marrow suppression (200 mCi)Pulmonary fibrosis Chronic sialadenitis, nodules, taste dysfunction Increased risk of cancer Anaplastic thyroid cancer Gastric cancer Hepatocellular cancer Lung cancer Breast cancer (>1000 mCi) Bladder cancer Hypoparathyroidism Slide 43 Prognosis Slide 44 Papillary Carcinoma >95% 10-yr survival AGES Scoring System (age, histologic grade, extra thyroidal invasion and metastasis, tumor size) MACIS (completeness of surgical resection, invasion and metastasis) AMES (age, metastasis, extra thyroidal spread and tumor size) TNM system Slide 45 Thyroid CA type5 year survival10 YEAR SURVIVAL Stage I Stage II Stage III Stage IV OVERALL PAPILLARY 100% 93%51%96-97%93% FOLLICULAR 100% 71%50%91%85% MEDULLARY 10098%81%28%80-86%75% ANAPLASTIC ---7%7-14%NO DATA Slide 46 Follicular Carcinoma Mortality: 15% at 10 years, 30% at 20 years Age > 50 at presentation, >4 cm, high tumor grade, vascular invasion, extrathyroidal invasion, distant mets POOR PROGNOSIS Slide 47 Medullary Carcinoma Depends on disease stage Approx. 80% 10-year survival rate 45% in patients with LN involvement Non-MEN (familial) >> MEN2A >> sporadic MTC >> MEN 2B Slide 48 Anaplastic Carcinoma Most aggressive Few patients survive 6 months after diagnosis