A Neuroendocrine Tumor Manifesting as a Gastrointestinal Catastrophe

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 306-326-Neoplasia of Endocrine Tissues: Case Reports
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-319
Farheen Kassim Dojki*1, Shounak Majumder1, Sidra Azim1, How Yee Heidi Hon1, Jorge Diez2 and James Feeney2
1University of Connecticut, Farmington, CT, 2Saint Francis Hospital and Medical Center, Hartford, CT
Multiple Endocrine Neoplasia 1 (MEN1) with associated gastrinoma is a rare clinical entity, which presents its own challenges in terms of timely diagnosis and treatment. A 63-year-old male presented with a two-year history of intermittent non-bloody diarrhea with worsening for 5 days associated with abdominal pain, nausea and weight loss. Past-Medical-History was significant for refractory Peptic-Ulcer-Disease and he denied any family history. Admission exam was unremarkable, however soon after he became hypotensive and developed lactic acidosis. Abdominal CT scan showed small retroperitoneal nodules and suspicion for bowel ischemia. He underwent removal of left ischemic colon. Post-operatively he had an episode of massive gastrointestinal-bleeding from the colostomy. Endoscopy revealed a large 2cm duodenal ulcer with active bleeding. After unsuccessful endoscopic hemostasis, he underwent surgical repair. Given his chronic diarrhea, duodenal ulcer and retro-pancreatic nodules, Zollinger-Ellison syndrome (ZES) was considered and fasting serum gastrin level was 6742 pg/mL (N <100 pg/ml). On re-exploration of the abdomen with intra-operative ultrasound, a 2cm nodule was resected from the pancreatic head and histopathological exam revealed a peripancreatic lymph node (LN) consistent with neuroendocrine tumor. Post-operatively gastrin levels declined to 235pg/ml and octreotide-scan was negative. Further evaluation revealed a 10mm non-functioning pituitary tumor supporting a diagnosis of sporadic MEN-1 as 2 out of the 3 glands were affected. Serum parathyroid hormone levels and parathyroid scan were normal. Genetic testing was not done. Gastrin-producing neuroendocrine tumors most commonly arise in the duodenum followed by the pancreas. The true incidence of ZES is unknown. Estimated incidence of gastrinomas in the United States ranges from 0.1-1% of patients with peptic-ulcer-disease. Compared to sporadic cases, gastrinomas in MEN1 are usually multifocal, smaller in size and difficult to localize, diagnose and treat as in our case. Primary lymph node gastrinoma is rare with less than 10% being found in the peripancreatic LN. Diagnostic criteria include (a) isolated LN involvement by tumor based on pre-operative imaging and intraoperative ultrasound and (b) disease-free status post-resection. Management of primary LN gastrinomas is controversial. To date there are no definitive studies to answer the need for prophylactic pancreaticoduodenectomy in MEN with gastrinoma. Isolated case reports favor LN resection followed by close follow-up as in our case. The clinical combination of chronic diarrhea and peptic-ulcer-disease should raise suspicion of gastrinoma. An elevated fasting serum gastrin level warrants MEN workup. Long-term follow-up data from large referral centers is needed to understand the natural history and optimize management of these patients.

1. C. Romei, E. Pardi, F. Cetani, and R. Elisei, "Genetic and Clinical Features of Multiple Endocrine Neoplasia Types 1 and 2", Journal of Oncology Volume 2012, Article ID 705036, Department of Endocrinology and Metabolism, University of Pisa, 56124 Pisa, Italy.further

Nothing to Disclose: FKD, SM, SA, HYHH, JD, JF

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