胃肠道小间质瘤研究进展.PDF

临床与病理杂志 2015, 35(1) J Clin Path ol R e s 155 doi: 10.3978/j.issn.2095-6959.2015.01.035 View this article at: /10.3978/j.issn.2095-6959.2015.01.035 胃肠道小间质瘤的研究进展 吴晶晶，张声 综述 (福建医科大学附属第一医院病理科，福州 350005) [摘　要] 胃肠道间质瘤(gastrointestinal stromal tumor ，GIST)是胃肠道最常见的间叶性肿瘤，约占胃肠道 间叶性肿瘤80% 。根据肿瘤大小、核分裂像计数以及肿瘤发生的部位将胃肠道间质瘤分为极低、 低、中等和高度危险性。体积 2 cm 的间质瘤，称为小间质瘤。不同危险度的间质瘤其临床表现、 生物学行为、遗传学表现和预后明显不同，小间质瘤的临床表现及预后明显好于大间质瘤。 C-Kit 基因突变是胃肠道间质瘤发生发展的驱动基因，因大小间质瘤 C-Kit基因突变相似，导致对 C-Kit基 因突变在胃肠道间质瘤中的作用引起争议。本文综述小间质瘤的流行病学、临床病理表现、分子 遗传学、治疗与预后的研究进展。 [关键词] 胃肠道间质瘤；小间质瘤；临床病理学；分子遗传学 Research advance of small gastrointestinal stromal tumors WU Jingjing, ZHANG Sheng (Department of Pathology, First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China) Abstract Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract that account for 80% of all GISTs. According to the tumor size, mitotic index and location, GISTs can be divide into very low, low, intermediate, high risk category, respectively. Small GISTs defined as less than 2.0 cm in diameter. The clinical manifestations, biology behavior, genetics and prognosis of the different risk GISTs are obviously different. Clinical study found that the clinical manifestation and prognosis of small GISTs are significantly better than GISTs. C-Kit mutations are the drive gene in the development of GISTs, the mutations are similar in small GISTs and overt GISTs, and so the role of the mutations is controversial. In t