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Case Reports in Surgery
Volume 2016, Article ID 1515202, 5 pages
http://dx.doi.org/10.1155/2016/1515202
Case Report

Multiple Gastric Gastrointestinal Stromal Tumors in a Patient with Neurofibromatosis Type 1

1Department of Surgery, Fujinomiya City General Hospital, 3-1 Nishiki-cho, Fujinomiya, Shizuoka 418-0076, Japan
2Department of Diagnostic Pathology, Fujinomiya City General Hospital, 3-1 Nishiki-cho, Fujinomiya, Shizuoka 418-0076, Japan
3Department of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan

Received 16 February 2016; Accepted 16 May 2016

Academic Editor: Tahsin Colak

Copyright © 2016 Makoto Tomatsu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Gastrointestinal stromal tumors (GISTs) are relatively common in neurofibromatosis type 1 (NF 1) patients. Approximately 90% of GISTs associated with NF 1 are located in the small intestine, while sporadic GISTs are most commonly located in the stomach. Here we report an extremely rare case of an NF 1 patient with multiple gastric GITs (90 or more) but without multiple small intestinal tumors. A 63-year-old female patient who had a history of NF 1 underwent surgery for a gastric neuroendocrine tumor and gastric submucosal tumor (SMT). During the operation, multiple small nodules were identified on the serosal surface of the upper stomach. SMT and multiple nodules on the serosal surface were diagnosed as GISTs consisting of spindle cells positive for KIT, CD34, and DOG-1. Both GIST and the normal gastric mucosa showed no mutations not only in the c-kit gene (exons 8, 9, 11, 13, and 17) but also in the PDGFRA gene (exons 12, 14, and 18). This patient is being followed up without the administration of a tyrosine kinase inhibitor.