Zhang Peng, Zeng Xiangyu, Gao Jinbo, Liu Weizhen, Shuai Xiaoming, Liu Ke, Liu Xinghua, Cai Ming, Cai Kailin, Wang Guobin, Tao Kaixiong
Department of Gastrointestinal Surgery, Union Hospital, Tongi Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.
Zhonghua Wei Chang Wai Ke Za Zhi. 2016 Nov 25;19(11):1290-1295.
To investigate the clinical characteristics, diagnosis and treatment as well as prognostic factors of the giant gastrointestinal stromal tumor (GIST).
Clinical data of 235 patients with high risk GIST treated in the Union Hospital, Tongi Medical College, Huazhong University of Science and Technology between January 2005 and July 2015 were retrospectively analyzed. Patients were divided into giant GIST group (diameter equal to or larger than 10 cm, 119 cases) and high risk group (diameter less than 10 cm, 116 cases) according to tumor size. Clinical characteristics and prognosis of two groups were compared and the clinical features of giant GIST were summarized. Multivariate analysis was performed to evaluate the prognostic factors of giant GIST with Cox regression model.
Of the 119 patients with giant GIST, which accounted for 50.6%(119/235) of all the high risk patients, there were 63 male and 56 female patients with a median age of 53(20-82) years. Primary giant GIST of 43(36.1%) located in the stomach, of 39(32.8%) in the small intestine, 5(4.2%) in the colon and rectum, and of 32 (26.9%) outside the gastrointestinal tract (mesentery, retroperitoneum, abdominal cavity, etc) and pelvic. Compared to high risk group, age of onset was younger [ratio of ≤50 years, 44.5%(53/119) vs. 31.9%(37/116), P = 0.046] and incidence of outside the gastrointestinal tract was significantly higher [26.9%(32/119) vs. 9.5%(11/116), P=0.000] in giant GIST group. All the giant GIST patients underwent surgical resection, including 115 cases(96.6%) of R0 resection, 3 cases(2.5%) of R1 resection and 1 case(0.9%) of R2 resection, besides, 32 cases(26.9%) underwent expanded resection (namely, underwent lymphadenectomy or combined organ resection simultaneously). Thirty-nine giant GIST cases(32.8%)accepted imatinib 400 mg/d for targeted therapy after operations, which was not significantly different with high risk group (46 cases, 39.6%, P=0.232). Relapse and metastasis occurred in 8 cases in giant GIST group. The 1-, 3-, 5-year overall survival rates of giant GIST group were 94.5%, 89.3%, 79.4% respectively and of high risk group were 99.1%, 92.9%, 85.1% respectively, and no significant difference was found (P=0.788). The 1-, 3-, 5-year recurrence-free survival rates of giant GIST group were 93.6%, 85.1%, 72.8% respectively and of high risk group were 99.1%, 91.7%, 84.2% respectively, and no significant difference was found as well (P=0.932). Multivariate analysis revealed that gender (P=0.047, RR=0.383, 95%CI:0.149-0.987), mitotic count (P=0.001, RR=0.216, 95%CI:0.087-0.538) and targeted therapy(P=0.019, RR=5.719, 95%CI:1.324-24.695) were prognostic risk factors of overall survival (OS), moreover, tumor size (P=0.024, RR=0.368, 95%CI:0.155-0.875) and mitotic count(P=0.007, RR=0.357, 95%CI:0.169-0.755) were prognostic risk factors of RFS.
Giant GIST is not unusual in GIST and more likely occurs outside gastrointestinal tract. Complete surgical excision combined with targeted therapy can improve the prognosis significantly. The prognosis of giant GIST and common high risk GIST is similar. Mitotic count is the most important prognostic factor.
探讨巨大胃肠道间质瘤（GIST）的临床特征、诊断治疗及预后因素。
回顾性分析2005年1月至2015年7月在华中科技大学同济医学院附属协和医院接受治疗的235例高危GIST患者的临床资料。根据肿瘤大小将患者分为巨大GIST组（直径大于或等于10 cm，119例）和高危组（直径小于10 cm，116例）。比较两组的临床特征及预后，并总结巨大GIST的临床特点。采用Cox回归模型进行多因素分析，评估巨大GIST的预后因素。
119例巨大GIST患者占所有高危患者的50.6%（119/235），其中男性63例，女性56例，中位年龄53（20 - 82）岁。43例（36.1%）原发性巨大GIST位于胃，39例（32.8%）位于小肠，5例（4.2%）位于结肠和直肠，32例（26.9%）位于胃肠道外（肠系膜、腹膜后、腹腔等）及盆腔。与高危组相比，巨大GIST组发病年龄较轻[≤50岁比例，44.5%（53/119）对31.9%（37/116），P = 0.046]，胃肠道外发生率显著更高[26.9%（32/119）对9.5%（11/116），P = 0.000]。所有巨大GIST患者均接受手术切除，其中R0切除115例（96.6%），R1切除3例（2.5%），R2切除1例（0.9%），此外，32例（26.9%）接受扩大切除（即同时行淋巴结清扫或联合器官切除）。39例巨大GIST患者（32.8%）术后接受伊马替尼400 mg/d靶向治疗，与高危组（46例，39.6%）相比差异无统计学意义（P = 0.232）。巨大GIST组8例出现复发转移。巨大GIST组患者1、3、5年总生存率分别为94.5%、89.3%、79.4%，高危组分别为99.1%、92.9%、85.1%，差异无统计学意义（P = 0.788）。巨大GIST组患者1、3、5年无复发生存率分别为93.6%、85.1%、72.8%，高危组分别为99.1%、91.7%、84.2%，差异也无统计学意义（P = 0.932）。多因素分析显示，性别（P = 0.047，RR = 0.383，95%CI：0.149 - 0.987）、核分裂象计数（P = 0.001，RR = 0.216，95%CI：0.087 - 0.538）和靶向治疗（P = 0.019，RR = 5.719，95%CI：1.324 - 24.695）是总生存（OS）的预后危险因素；此外，肿瘤大小（P = 0.024，RR = 0.368，95%CI：0.155 - 0.875）和核分裂象计数（P = 0.007，RR = 0.357，95%CI：0.169 - 0.755）是无复发生存（RFS）的预后危险因素。
巨大GIST在GIST中并不少见，且更易发生于胃肠道外。完整手术切除联合靶向治疗可显著改善预后。巨大GIST与普通高危GIST的预后相似。核分裂象计数是最重要的预后因素。
