Concomitant Carcinoid, Gastrointestinal Stromal Tumor and Ganglioneuroma in a Male with Abdominal Pain

Abstract

Purpose: Concomitant presentation of Carcinoid, Gastrointestinal Stromal Tumor (GIST) and Ganglioneuroma (GN) has not yet been reported. Due to its novelty, screening, management, and prognosis of tri-concomitant malignancies involving carcinoid tumor is unknown. We describe a 51-year-old male with no significant medical history who initially presented to the PCP for diffuse intermittent abdominal pain. He was subsequently referred to Gastroenterology and underwent a screening colonoscopy which showed superficial lobular changes at the proximal colon. Immunostain analysis on biopsy specimen was positive for chromogranin and synaptophysin confirming Carcinoid tumor. Imaging studies for staging revealed two hepatic lesions that were positive on Octreotide scan. He subsequently underwent exploratory laparotomy, right hemicolectomy, resection of the small bowel and jejunal tumors and liver wedge resection. Pathologic findings on resected small bowel identified a Gastrointestinal stromal tumor (GIST), while the right colon and distal ileum showed Carcinoid tumor with liver metastasis. Furthermore, a solitary Ganglioneuroma was identified within the colon. To our knowledge, synchronous presentation of Carcinoid, GIST and Ganglioneuroma has not yet been reported, and a thorough literature search failed to yield any such cases. Pathogenesis of concomitant presentation within this patient remains unclear. One could speculate that the occurrence of these neoplasms may involve common oncogenic pathways that are yet to be discovered, or alternatively carcinoid tumor render other cell types susceptible for malignancies. The reported increased incidence of other malignancies in carcinoid patients begs the question whether a thorough screening protocol should be instituted at time of carcinoid diagnosis. Furthermore, does synchronous presentation affect prognosis that may perhaps influence management?