囊性胃肠间质瘤的临床病理特征及预后  

Clinicopathological features and prognosis of cystic gastrointestinal stromal tumor

作　　者：王翠众[1] 侯英勇[2] 沈坤堂[1] 王洪山[1] 秦净[1] 孙益红[1] 秦新裕[1] 

机构地区：[1]复旦大学附属中山医院普通外科,上海200032 [2]复旦大学附属中山医院病理科,上海200032

出　　处：《中华胃肠外科杂志》2011年第8期599-602,共4页Chinese Journal of Gastrointestinal Surgery

基　　金：基金项目：上海市浦江人才计划（09PJD009）

摘　　要：目的探讨囊性胃肠间质瘤（GIST）的临床病理学和分子遗传学特征，并评估其生物学行为。方法对复旦大学附属中山医院2005年2月至2010年1月间收治的7例以囊性结构为主的GIST病例的临床资料进行回顾性分析，并检测其c—kit和PDGFR-α基因突变状况。结果7例患者中男性2例，女性5例；年龄为46。76岁；肿瘤来源于胃4例，十二指肠1例，小肠2例；肿瘤大小6～16cm．瘤体显著囊性变。镜下实性区可见肿瘤细胞，其中4例为上皮样细胞型．3例为梭形细胞型．核分裂像0～3个／50HPF。按照美国国立卫生研究院（NIH）危险度分级，4例为高度危险，3例为中度危险：但依据形态学特点，3例为交界性，3例为低度恶性，1例为中度恶性。3例患者检测到c—kit基因第11外显子突变。经9～80个月的随访．7例患者均为无瘤生存．无一例出现复发或转移。结论囊性GIST临床表现为惰性生物学行为．恶性风险低。Objective To investigate the clinicopathological and molecular genetic characteristics of gastrointestinal stromal tumor （GISTs） with significant cystic changes, and to assess their biological behavior. Methods Clinicopathological features of 7 patients with cystic GISTs treated at the Zhongshan Hospital of Fudan University from February 2005 to January 2010 were summarized retrospectively. The mutations status of c-kit and PDGFR-α were analyzed. Results There were 2 males and 5 females aged from 46 to 76 years old. Primary site of GISTs included stomach （n=4）, duodenum （n=1）, and small intestinal （n=2）. Tumor size ranged from 6 to 16 cm with obviously cystic changes. Tumor cells were found in the solid components under microscope, of which epithelioid cell type were found in 4 case and spindle cell type in 3 cases. The mitotic figures were no more than 3/50 HPF in all the patients. According to the NIH criteria, 4 were high-risk and 3 were low-risk. Based on morphological characteristics, 3 cases were as borderline tumor, 3 moderate-risk, and 1 moderate-risk. Gene mutation of exon 11 of c-kit were identified in 3 cases. During the follow up ranging from 9 to 80 months, all the 7 patients had cancer-free survival. Conclusion The biological behavior of cystic GIST is indolent with a low risk of malignancy and favorable prognosis.

关 键 词：胃肠间质瘤 囊性样变 临床病理特征 预后 

分 类 号：R735[医药卫生—肿瘤]