S2820 Perforated Ulcer as a Complication of Duodenal Neuroendocrine Tumor

Abstract

INTRODUCTION: The incidence of duodenal neuroendocrine tumors (D-NETs) is only 0.19 per 100,000 in the US. Very little is known about the various patient presentations, and therefore every case is valuable. We present a patient found to have a duodenal NET and duodenal peptic ulcer disease complicated by sudden perforation. CASE DESCRIPTION/METHODS: A 54 year old female with lupus, antiphospholipid syndrome, cerebrovascular accident, heart failure, and left ventricular thrombus on warfarin, presented with melena, non bloody non bilious vomiting and a hemoglobin of 6. EGD revealed a duodenal 4 mm nodule consistent with well differentiated NET on histopathology and multiple 2 mm clean base ulcers. The patient was transfused blood and treated with PPI therapy. Serotonin and gastrin levels were < 20 and 60 respectively. Chromogranin A level was elevated at 1486. She was scheduled for EUS guided endoscopic resection of her NET. She later developed acute abdominal pain and coffee ground emesis. CT Angiography was negative for mesenteric ischemia but revealed pneumoperitoneum and hemorrhagic ascites. She underwent emergency exploratory laparotomy with repair of perforation of duodenal wall but decompensated and expired. DISCUSSION: The WHO classifies NETs based on mitotic activity, G1 to G3. D-NET can also be classified as gastrinomas (50–60%), somatostatinomas (15%), nonfunctional tumors (19-27%), paragangliomas (< 2%) and poorly differentiated carcinomas (< 3%). Diagnosis is usually incidental during EGD evaluation and is based on histological analysis for chromogranin and synaptophysin. Our patient was on anticoagulation and presented with peptic ulcer disease and NET in the duodenum. Gastrin level was only 60, ruling out a functional gastrinoma and Zollinger Ellison Syndrome. 80% of NETs tend to have elevated serum chromogranin. Only 3% of patients develop carcinoid syndrome, a complication of metastasis to the liver. For our patient, CT did not reveal any metastasis and serotonin levels were normal. Local nonfunctional < 1 cm NET can be endoscopically resected. Our patient was planned for EUS guided resection, but she did not make it to her procedure. EGD showed clean based ulcers, which have the lowest risk for ulcer complications. It is reasonable to speculate that her NET may have contributed to mucosal fragmentation and ulcer perforation. Unusual presentations of rare diseases such as D-NETs should be reported and is an area that requires further research.