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제1형 신경섬유종증 환자에게서 함께 발견된 위장관 기질종양과 십이지장 팽대부 신경내분비 종양: 3예의 증례

Synchronous Gastrointestinal Stromal Tumor and Ampullary Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Report of Three Cases

대한소화기학회지 2019년 74권 4호 p.227 ~ 231
박은규 ( Park Eun-kyu ) - 전남대학교병원 외과

김희준 ( Kim Hee-Joon ) - 전남대학교병원 외과
이윤호 ( Lee Yun-Ho ) - 전남대학교병원 외과
고양석 ( Koh Yang-Seok ) - 전남대학교 의과대학 화순전남대학교병원 외과
허영회 ( Hur Young-Hoe ) - 전남대학교 의과대학 화순전남대학교병원 외과
조철균 ( Cho Chol-Kyoon ) - 전남대학교 의과대학 화순전남대학교병원 외과

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.

키워드

Neurofibromatosis 1; Neuroendocrine tumors; Gastrointestinal stromal tumors; Surgical treatment
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