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Korean J Gastroenterol  <  Volume 74(4); 2019 <  Articles

Korean J Gastroenterol 2019; 74(4): 227-231  https://doi.org/10.4166/kjg.2019.74.4.227
Synchronous Gastrointestinal Stromal Tumor and Ampullary Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Report of Three Cases
Eun Kyu Park, Hee Joon Kim, Yun Ho Lee, Yang Seok Koh1, Young Hoe Hur1 and Chol Kyoon Cho1
Department of Surgery, Chonnam National University Hospital, Gwangju; Department of Surgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School1, Hwasun, Korea
Correspondence to: Chol Kyoon Cho, Department of Surgery, Chonnam National University Medical School, 160 Baekseo-ro, Dong-gu, Gwangju 61469, Korea. Tel: +82-62-220-6451, Fax: +82-62-227-1635, E-mail: ckcho@jnu.ac.kr, ORCID: https://orcid.org/0000-0001-8457-7314
Received: July 10, 2019; Revised: August 2, 2019; Accepted: August 5, 2019; Published online: October 25, 2019.
© The Korean Journal of Gastroenterology. All rights reserved.

This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disorder. The pathogenesis of NF1 is suggested to be an alteration of the NF-1 gene, which normally functions as a tumor suppressor. A mutation of NF-1 causes the development of viable tumors in various sites. On the other hand, the synchronous manifestation of a gastrointestinal stromal tumor (GIST) and neuroendocrine tumor (NET) in the background of NF1 is extremely rare. This paper reports three cases treated with surgical intervention along with the long-term follow-up results. Three patients showed synchronous ampullary NET and GIST in association with NF1 supported by postoperative histopathologic analysis. Surgical treatments, such as pancreatoduodenectomy and local excision were applied. No recurrence occurred during the postoperative follow-up period of 10, 9, and 2.7 years. Synchronous GIST and NET in the background of NF1 is extremely rare, but the possible coexistence of other tumors in NF1 patients is relatively higher than that in the general population. Furthermore, both NETs and GISTs occurring in NF1 patients tend to be smaller in size compared to that in the general population. Therefore, when NF1 patients present with vague abdominal discomfort, close attention must be paid to identifying the coexistence of other neoplasms.
Keywords: Neurofibromatosis 1; Neuroendocrine tumors; Gastrointestinal stromal tumors; Surgical treatment


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