3
stomach. It should be surrounded by a coat of shared smooth muscle. A common blood supply should be pres- ent. It should be lined by alimentary-tract epithelium. Rarely, respiratory-tract epithelium, in conjunction with ali- mentary-tract epithelium, has been noted. 3,4 In adults, diagnosis is very difficult before surgery. Gastric duplication cysts are usually asymptomatic. 5,6 However, signs and symptoms may include epigastric pain, abdominal mass, or vomiting. Rarely, GI bleeding, pancreatitis, peritoni- tis, malignancy, or even acute abdomen may result. 6-8 Often, a GDC may be mistaken as a pancreatic pseudocyst, which is more common. 8 Normal levels of pancreatic enzymes and no personal history of pancreatitis may suggest a gastric duplication cyst. Unlike GDC, the pancreatic pseudocyst is generally associated with repeated attacks of pancreatitis. They lack an epithelial lining and contain amylase-rich fluid. 8 US, CT, and EUS may help in the diagnosis. In our case, the diagnosis was made before surgery by EUS-guided cyst aspiration and ERP. Surgical resection led to a satisfactory outcome. This case provides a fascinating opportunity to explore embryogenesis of the alimentary tract, as well as highlighting the usefulness of EUS and ERP in preoperative diagnosis. DISCLOSURE All authors disclosed no financial relationships rele- vant to this publication. Abbreviations: ERP, endoscopic retrograde pancreatography; GDCs, gastric duplication cysts. REFERENCES 1. Muraoka A, Tsuruno M, Katsuno G, et al. A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case. Surg Today 2002;32:531-5. 2. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia in a gastric duplication cyst: a case report and review of the literature. Eur J Pediatr Surg 2004;14:60-2. 3. O’Donnell PL, Morrow JB, Fitzgerald TL. Adult gastric duplication cysts: a case report and review of literature. Am Surg 2005;71:522-5. 4. Takahara T, Torigoe T, Haga H, et al. Gastric duplication cyst: evaluation by endoscopic ultrasonography and magnetic resonance imaging. J Gastroenterol 1996;31:420-4. 5. Johnstone DW, Forde KA, Markowitz D, et al. Gastric duplication cyst communicating with the pancreatic duct: a rare cause of recurrent abdominal pain. Surgery 1991;109:97-100. 6. Coit DG, Mies C. Adenocarcinoma arising within a gastric duplication cyst. J Surg Oncol 1992;50:274-7. 7. Horne G, Ming-Lum C, Kirkpatrick AW, et al. High-grade neuroendocrine carcinoma arising in a gastric duplication cyst: a case report with liter- ature review. Int J Surg Pathol 2007;15:187-91. 8. Yang MC, Duh YC, Lai H, et al. Alimentary tract duplications. J Formos Med Assoc 1996;95:406-9. Current affiliations: Departments of Medicine (T.S.), Pathology (J.L., J.S.), Gastroenterology (R.A. M.D.), Allegheny General Hospital, Pittsburgh, Pennsylvania, USA. Reprint requests: Trupti Shinde, MD, Internal Medicine, Allegheny General Hospital, Medicine Department, 320 E. North Ave, Pittsburgh, PA 15212. Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy 0016-5107/$36.00 doi:10.1016/j.gie.2008.04.059 Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association Jayaprakash Sreenarasimhaiah, MD, Luis A. Armstrong, MD, Shou-Jiang Tang, MD, Carlton Barnett, MD Dallas, Texas, USA Tuberous sclerosis is an autosomal dominant condition characterized by the triad of mental deficiency, epilepsy, and the classic skin eruption known as adenoma seba- ceum. 1 Other findings include visceral lesions with histol- ogy similar to the skin lesions, renal angiomyolipomas, and pancreatic abnormalities. Findings in the pancreas can include pancreatic cysts, rare angiomyolipomas, and neuroendocrine tumors of the nonfunctioning islet cell variety. 2 Results of certain reports suggest that functional and nonfunctional insulinomas may be associated with this disorder. To our knowledge, this is the first reported case of pancreatic somatostatinoma associated with tuber- ous sclerosis. CASE REPORT A 24-year-old man with a history of tuberous sclerosis complained of 3 months of dull abdominal pain. He denied symptoms of GI bleeding, jaundice, nausea, or vomiting, or association with food intake. He reported a mild weight loss of 10 pounds during this period. His history also included www.giejournal.org Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 379 Brief Reports

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Page 1: Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association

stomach. It should be surrounded by a coat of sharedsmooth muscle. A common blood supply should be pres-ent. It should be lined by alimentary-tract epithelium.Rarely, respiratory-tract epithelium, in conjunction with ali-mentary-tract epithelium, has been noted.3,4

In adults, diagnosis is very difficult before surgery. Gastricduplication cysts are usually asymptomatic.5,6 However,signs and symptoms may include epigastric pain, abdominalmass, or vomiting. Rarely, GI bleeding, pancreatitis, peritoni-tis, malignancy, or even acute abdomen may result.6-8 Often,a GDC may be mistaken as a pancreatic pseudocyst, which ismore common.8 Normal levels of pancreatic enzymes andno personal history of pancreatitis may suggest a gastricduplication cyst. Unlike GDC, the pancreatic pseudocyst isgenerally associated with repeated attacks of pancreatitis.They lack an epithelial lining and contain amylase-rich fluid.8

US, CT, and EUS may help in the diagnosis.In our case, the diagnosis was made before surgery by

EUS-guided cyst aspiration and ERP. Surgical resection ledto a satisfactory outcome. This case provides a fascinatingopportunity to explore embryogenesis of the alimentarytract, as well as highlighting the usefulness of EUS andERP in preoperative diagnosis.

DISCLOSURE

All authors disclosed no financial relationships rele-vant to this publication.

Abbreviations: ERP, endoscopic retrograde pancreatography; GDCs,

gastric duplication cysts.

REFERENCES

1. Muraoka A, Tsuruno M, Katsuno G, et al. A gastric duplication cyst with

an aberrant pancreatic ductal system: report of a case. Surg Today

2002;32:531-5.

2. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia

in a gastric duplication cyst: a case report and review of the literature.

Eur J Pediatr Surg 2004;14:60-2.

3. O’Donnell PL, Morrow JB, Fitzgerald TL. Adult gastric duplication cysts:

a case report and review of literature. Am Surg 2005;71:522-5.

4. Takahara T, Torigoe T, Haga H, et al. Gastric duplication cyst: evaluation

by endoscopic ultrasonography and magnetic resonance imaging.

J Gastroenterol 1996;31:420-4.

5. Johnstone DW, Forde KA, Markowitz D, et al. Gastric duplication cyst

communicating with the pancreatic duct: a rare cause of recurrent

abdominal pain. Surgery 1991;109:97-100.

6. Coit DG, Mies C. Adenocarcinoma arising within a gastric duplication

cyst. J Surg Oncol 1992;50:274-7.

7. Horne G, Ming-Lum C, Kirkpatrick AW, et al. High-grade neuroendocrine

carcinoma arising in a gastric duplication cyst: a case report with liter-

ature review. Int J Surg Pathol 2007;15:187-91.

8. Yang MC, Duh YC, Lai H, et al. Alimentary tract duplications. J Formos

Med Assoc 1996;95:406-9.

Current affiliations: Departments of Medicine (T.S.), Pathology (J.L., J.S.),

Gastroenterology (R.A. M.D.), Allegheny General Hospital,

Pittsburgh, Pennsylvania, USA.

Reprint requests: Trupti Shinde, MD, Internal Medicine, Allegheny General

Hospital, Medicine Department, 320 E. North Ave, Pittsburgh, PA 15212.

Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy

0016-5107/$36.00

doi:10.1016/j.gie.2008.04.059

Brief Reports

Pancreatic somatostatinoma and tuberous sclerosis: case report ofan exceedingly rare association

Jayaprakash Sreenarasimhaiah, MD, Luis A. Armstrong, MD, Shou-Jiang Tang, MD,Carlton Barnett, MD

Dallas, Texas, USA

Tuberous sclerosis is an autosomal dominant conditioncharacterized by the triad of mental deficiency, epilepsy,and the classic skin eruption known as adenoma seba-ceum.1 Other findings include visceral lesions with histol-ogy similar to the skin lesions, renal angiomyolipomas,and pancreatic abnormalities. Findings in the pancreascan include pancreatic cysts, rare angiomyolipomas, andneuroendocrine tumors of the nonfunctioning islet cellvariety.2 Results of certain reports suggest that functionaland nonfunctional insulinomas may be associated withthis disorder. To our knowledge, this is the first reported

case of pancreatic somatostatinoma associated with tuber-ous sclerosis.

CASE REPORT

A 24-year-old man with a history of tuberous sclerosiscomplained of 3 months of dull abdominal pain. He deniedsymptoms of GI bleeding, jaundice, nausea, or vomiting, orassociation with food intake. He reported a mild weight lossof 10 pounds during this period. His history also included

www.giejournal.org Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 379

Page 2: Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association

Brief Reports

multiple renal angiomyolipomas, acute myelocytic leuke-mia, and seizures. A family history of GI malignancies wasnot present.

A physical examination revealed multiple raised lesionson the nose, forehead, and upper cheeks bilaterally(Fig. 1). An abdominal examination was unremarkable fororganomegaly, Murphy’s sign, or palpable masses. Chemis-try and hematologic laboratory examinations showed nor-mal results, including serum chromogranin A (89 ng/mL;normal !225 ng/mL). The patient underwent a CT, whichdemonstrated multiple stable lesions in the kidneys bilater-ally compared with prior studies. A 4.8-cm� 4.2-cm homog-enous mass was noted in the head and uncinate process ofthe pancreas (Fig. 2). Detailed evaluation of the tumor wasperformed by EUS to determine vascular or lymph-nodeinvasion, as well as to obtain a histologic diagnosis

Figure 2. CT of pancreas mass.

Figure 1. Cutaneous findings of tuberous sclerosis.

380 GASTROINTESTINAL ENDOSCOPY Volume 69, No. 2 : 2009

(Fig. 3). This study revealed a well-defined hypodenselesion, without pancreatic-duct or bile-duct dilatation. Thelesion appeared to be in close proximity to the superiormesenteric vein, without invasion. FNAs were performedwith a 19-gauge needle. Cell-block preparations showedclusters of neuroendocrine-appearing cells characterizedby abundant eosinophilic cytoplasm, with eccentricallyplaced bland, oval nuclei, and only mild nuclear pleomor-phism (Fig. 4). Immunostains were positive for somato-statin (Fig. 5), synaptophysin (Fig. 6), chromogranin, andKi-67. Stains were negative for pancreatic polypeptide, insu-lin, gastrin, villin, human chorionic gonadotropin, and tran-scription termination factor-1. Serum somatostatin levelwas 15 pg/dL (reference range!25 pg/dL), which suggesteda nonfunctional tumor. Moreover, the patient had no signsof cholelithiasis, hyperglycemia, or steatorrhea.

The patient underwent surgical exploration, with subse-quent Whipple pancreaticoduodenectomy. A small 1-cmlesion of uncertain significance was seen in the right lobeof the liver and was ablated by alcohol injection. Tumor

Figure 3. EUS image of pancreas mass.

Figure 4. Pancreas mass biopsy (H&E, orig. mag. �100).

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Page 3: Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association

Brief Reports

margins were adequate, and lymph-node dissection wasnegative for malignant spread. The patient was doing wellat 4 months of follow-up.

DISCUSSION

Neuroendocrine tumors of the pancreas are often inci-dentally found during radiographic imaging of the abdo-men for other purposes. However, results of severalstudies suggest that the incidence of such tumors isincreased in the setting of tuberous sclerosis but remainsrare. Results of some reports suggest an increased

frequency of multiple endocrine neoplasia (MEN 1) and gas-trinoma or insulinoma in these patients.3

Somatostatinoma is a rare endocrine tumor that occurswith similar frequency in both the duodenum and the pan-creas. Many of these somatostatinomas are sporadic butmay be detected in association with neurofibromatosistype 1, MEN 1, and von Hippel-Lindau syndromes. The clas-sic clinical triad of functional somatostatinomas includesmild diabetes, cholelithiasis, and steatorrhea, althoughthe majority of tumors are nonfunctioning.4 These tumorsare usually identified as large lesions when found in the pan-creas and are metastatic in 70% of cases at the time of diag-nosis.5 The setting of tuberous sclerosis should promptevaluation of the pancreas in the hopes of early detectionof neuroendocrine tumors and favorable therapeutic out-comes for these individuals. Among neuroendocrinetumors in this setting, somatostatinoma is exceedingly rare,as this case illustrates.

DISCLOSURE

All authors disclosed no financial relationships rele-vant to this publication.

Abbreviation: MEN, multiple endocrine neoplasia.

REFERENCES

1. Rosser T, Panigrahy A, McClintock W. The diverse clinical manifestations

of tuberous sclerosis complex: a review. Semin Pediatr Neurol 2006;13:

27-36.

2. Alexakis N, Connor S, Ghaneh P, et al. Hereditary pancreatic endocrine

tumours. Pancreatology 2004;4:417-33.

3. Eledrisi MS, Stuart CA, Alshanti M. Insulinoma in a patient with tuberous

sclerosis: is there an association? Endocr Pract 2002;8:109-12.

4. Moayedoddin B, Booya F, Wermers RA, et al. Spectrum of malignant

somatostatin-producing neuroendocrine tumors. Endocr Pract 2006;

12:394-400.

5. Stephen AE, Hodin RA. Neuroendocrine tumors of the pancreas, excluding

gastrinoma. Surg Oncol Clin N Am 2006;15:497-510.

Current affiliations: Division of Digestive and Liver Diseases (J.S., L.A.A.,

S.-J.T.), Department of Medicine, Department of Surgery (C.B.), University

of Texas Southwestern Medical Center, Dallas, Texas, USA.

Reprint requests: Jayaprakash Sreenarasimhaiah, MD, Division of

Digestive and Liver Diseases, University of Texas Southwestern Medical

Center, 1801 Inwood Rd, Ste 6.102 MC 9083, Dallas, TX 75390.

Copyright ª 2009 by the American Society for Gastrointestinal Endoscopy

0016-5107/$36.00

doi:10.1016/j.gie.2008.04.057

Figure 5. Pancreas mass biopsy (somatostatin immunostain, orig. mag.

�200).

Figure 6. Pancreas mass biopsy (synaptophysin immunostain, orig. mag.

�200).

www.giejournal.org Volume 69, No. 2 : 2009 GASTROINTESTINAL ENDOSCOPY 381