Agarwal Rinki, Robson Mark
Department of Medicine, Clinical Genetics, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
Hematol Oncol Clin North Am. 2009 Feb;23(1):1-13, vii. doi: 10.1016/j.hoc.2008.12.003.
Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract and are a relatively recently identified category of mesenchymal tumors. Germline mutations in a number of different genes predispose to GIST. This article discusses familial GIST syndrome, Carney triad, Carney-Stratakis syndrome, and neurofibromatosis type 1, and addresses the recognition of an inherited predisposition in GIST patients.
胃肠道间质瘤（GIST）是胃肠道最常见的间叶组织肿瘤，是一类相对较新发现的间叶组织肿瘤。多种不同基因的种系突变易引发GIST。本文讨论了家族性GIST综合征、卡尼三联征、卡尼-斯特拉塔基斯综合征和1型神经纤维瘤病，并阐述了对GIST患者遗传性易感性的认识。
