Objective: This case serves as an uncommon presentation of carcinoid tumor diagnosed in a patient with history of Ulcerative Colitis. To date, only 50 cases of carcinoid tumor with IBD have been reported since 1942, with only two were diagnosed pre-operatively. Methods: This case is based on an extensive literature review involving minimal case reports and case series. Case Presentation: A 46-year old man with long standing history of ulcerative colitis developed refractory disease and underwent total proctocolectomy with ileo-anal–J pouch. Three years after the surgery, he presented with symptoms of fatigue, dizziness and dyspnea on exertion lasting one week. He also reported melanotic stools and pica. Hemoglobin on presentation was 7.7g/dl, despite previous normal values. He underwent an EGD showing a 2 cm submucosal nodule in the duodenal bulb, and later EUS with biopsy. Both pathologic specimens were positive for well-differentiated neuroendocrine tumor (carcinoid), and IHC stains were positive for gastrin, but negative for Somatostatin. The tumor stains were also positive for Chromogranin and Synaptophysin. The patient then underwent pouchoscopy and capsule endoscopy which found mild ulceration in the distal bowel. The imaging studies, including CT abdomen as well as an Octreotide scan, did not reveal evidence of metastases. He subsequently underwent resection of the tumor with pathology confirming a stage II-A, low-grade neuroendocrine carcinoid tumor with a proliferative index Ki-67 of 1%. Hemoglobin levels returned to baseline within two months and the patient continues to do well clinically. Discussion: We are reporting a rare presentation of a carcinoid tumor diagnosed pre-operatively by biopsy in a patient with Inflammatory Bowel Disease. In IBD, enteroendocrine cells can be hyper-stimulated by inflammation to cause hyperplasia and neoplasia, although the time period from conversion of entero-chromaffin like cell (ECL) hyperplasia to ECL like Carcinoids is unclear. Interestingly, most of the case reports in the literature have documented Carcinoid tumors in the un-inflamed intestine. While this inflammatory mechanism is a theoretically justifiable concern, aberrant endocrine cells have not been definitively linked to the presence of carcinoid tumors. Therefore, the association of carcinoid and IBD awaits confirmation. Conclusion: Based on literature review, there have been only two other cases involving diagnosis of a carcinoid tumor pre-operatively. With these case reports it is difficult to determine if the association between them is fortuitous, or if in fact IBD is really a risk factor for the tumor. More studies are needed to known if ECL like hyperplasia plays any role to Carcinoid tumor with IBD.
Mankala, S. Matin, A. Desai, D. Shaik, S. Prutzman, D. Bhatt, B. (2016, May). IBD with a Side of Carcinoid. Poster Presented at: (AACE) The American Association of Clinical Endocrinologists, Orlando, FL.