Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.

Abstract
Neurofibromatosis type‑1 (NF‑1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50‑year‑old female NF‑1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Thorough radiological and peroperative work‑up revealed multiple ulcerated submucosal and serosal nodules in the proximal small intestine. Histological work‑up revealed diagnosis of a duodenal submucosal somatostatinoma with multifocal serosal gastrointestinal stromal tumors. This case is being reported to highlight the rare coincidence of multiple GIT tumors in an NF‑1 patient.
Description
Keywords
Multiple gastrointestinal stromal tumors, neurofibromatosis type 1, periampullary somatostatinoma
Citation
Kumar Tarun, Gupta Brijnandan, Das Prasenjit, Jain Deepali, Jain Hemant Ashok, Madhusudhan Kumble S, Dash Nihar Ranjan, Gupta Siddhartha Datta. Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1. Indian Journal of Pathology & Microbiology. 2016 July-Sept 59(3): 359-361.