2524 Rare Case of Numerous Duodenal Neuroendocrine Tumors Encompassing Entire Proximal Duodenum

Abstract

INTRODUCTION: Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, but incidence is on the rise, in part due to increased detection. GI NETs can be found from the esophagus to the rectum. The appendix is the most common location.1,2 Primary duodenal NETs (D-Nets) are rare and account for less than 2% of tumors1. We report a case of a rare multiple duodenal NET. CASE DESCRIPTION/METHODS: 44-year-old female who presented for anemia complaining of belching, flatus, and loose stools. EGD found the post-bulbar area studded with numerous 1 cm nodules, covering the entire proximal colon. Endoscopic biopsy revealed a spectrum of proliferations, which followed the distribution of Brunner glands. Many of the proliferations turned in the full-blown D-NETs positive for synaptophysin and chromogranin, the Ki-67 index was less than 3%, consent with neuroendocrine neoplasm grade 1 (NET tumor), in the 1st through the 3rd portion of the duodenum. Octrotide scan was negative. The patient was sent for a Whipple procedure. MEN type 1, NF, TSH, and 24 hr urine 5-HIAA were normal. DISCUSSION: GI NETs are often diagnosed in the 5th–6th decade of life and are common in African Americans and females.3 Primary tumors in the duodenum are rare and their frequency decreases toward the distal duodenum.4 Symptoms are non-specific, presenting with dyspepsia and epigastric pain. EGD is the best method for preoperative diagnosis, neuroendocrine markers such as serum chromogranin A and urine 5-HIAA, are supportive.4 In duodenal carcinoid tumors, ∼4% are associated with carcinoid syndrome.5 Duodenal carcinoids can be associated with clinical syndromes, such as Zollinger-Ellison syndrome or Multiple endocrine neoplasia-1 3. Carcinoid tumors, with metastasis express, have a high affinity for octreotide, and can be visualized with an OctreoScan.2,3 Duodenal tumors can be removed endoscopically or with resection. Our patient underwent Whipple's pancreaticoduodenectomy due to the peri-ampullary location of the tumors (Figure 1). The current study describes a rare case of multiple D-NETs carpeting the proximal duodenum, in a patient without elevated neuroendocrine markers, ZES, or MEN. Proliferation in the distribution of the Brunner glands indicates an underlying genetic alteration in the neuroendocrine cells associated with THE Brunner glands. From our literature search, we were only able to find two other case reporting multiple D-NETs and no cases reporting D-NETs proliferations from Brunner glands, thus a rare presentation.