Clinical characteristics and prognosis of primary gastrointestinal stromal tumors with PDGFRα mutation
10.3760/cma.j.cn113855-20200313-00193
- VernacularTitle:原发血小板源性生长因子受体α多肽基因突变型胃肠道间质瘤的临床病理特征及预后分析
- Author:
Weizhen LIU
1
;
Tao WANG
;
Peng ZHANG
;
Xin CHEN
;
Xiong SUN
;
Yao LIN
;
Wenze WAN
;
Xinghua LIU
;
Guobin WANG
;
Kaixiong TAO
Author Information
1. 华中科技大学同济医学院附属协和医院胃肠外科，武汉 430022
- From:
Chinese Journal of General Surgery
2020;35(8):624-627
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinicopathological characteristics of primary gastrointestinal stromal tumors (GIST) with PDGFRα mutation and analyze the prognosis of different subtypes.Methods:From Jun 2010 to Jun 2019, the clinicopathological data of 35 patients with primary PDGFRα mutation GIST, who underwent surgical therapy in the Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, were analyzed retrospectively.Results:The main symptoms was abdominal pain (28 cases, 80%), followed by abdominal mass (6 cases, 17%), and hemafecia (1 case, 3%). 31 primary lesions (89%) were located in the stomach and 4 (11%) in other than stomach. 13 cases (37%) were of epithelioid cells, 14 cases (40%) were of spindle cells and 8 cases (23%) were of mixed cells. 27 cases (77%) were CD117 positive , 28 cases (80%) CD34 positive , and 30 cases (86%) were DOG-1 positive. 19 cases (54%) had D842V mutation and 16 cases (46%) had non-D842V mutation. Complete surgical resection was performed in all patients, with no perioperative death. The 3-year recurrence-free survival rate of the D842V mutation group was lower than that of the non-D842V mutation group (84% vs. 100%, P=0.045). Conclusions:The mutation rate of PDGFRα gene was low, mostly derived from the stomach. PDGFRα mutation GIST presents inert biological behavior and the overall prognosis was good.
