目的：探讨巨大胃肠道间质瘤（GIST）的临床特征、诊断治疗方法及预后。方法：回顾性分析2012 年1 月—2015 年4 月间在川北医学院附属医院收治的179 例GIST 患者资料，其中39 例为巨大GIST（直径≥ 10 cm）。结果：39 例巨大GIST 患者的发病部位分别为胃部14 例（35.6%）、消化道外（肠系膜、网膜、腹膜）10 例（25.6%）、回肠7 例（17.9%）、空肠4 例（10.3%）、十二指肠3 例（7.7%）、直肠1 例（2.6%）；与普通GIST（直径<10 cm）比较，巨大GIST 患者中核分裂像、包膜破损、多发、胃肠道外GIST、术前贫血及并发症比例明显增高（均P<0.05）。39 例患者中，单纯性肿瘤切除8 例（20.5%），合并胃肠等器官部分切除30 例（76.9%），1 例（2.6%）肿瘤未能切除；39 例巨大GIST 均为高危风险度，32 例患者获得有效随访，4 例（10.3%）术后服用伊马替尼治疗；术后1、2 年无进展生存率分别为92.8%、79.6%。结论：巨大GIST 可根据临床表现、影像学及病理检查结果诊断，其临床特征表现出较高的危险度，完整肿瘤切除合并胃肠等器官部分切除以及规范的术前与术后靶向治疗是改善患者预后的有效手段。
Giant gastrointestinal stromal tumors: a clinical analysis of 39 cases
Objective: To investigate the clinical features, diagnosis and treatment methods as well as prognosis of the giant gastrointestinal stromal tumor (GIST). Methods: The clinical data of 179 patients with GIST treated in the Affiliated Hospital of North Sichuan Medical College from January 2012 to April 2015 were retrospectively analyzed, of whom 39 cases had giant GIST (diameter equal to or larger than 10 cm). Results: Of the 39 patients with giant GIST, the tumors occurred in the stomach in 14 cases (35.6%), extragastrointestinal locations (mesentery, omentum and peritoneum) in 10 cases (25.6%), ileum in 7 cases (17.9%), jejunum in 4 cases (10.3%), duodenum in 3 cases (7.7%), and rectum in 1 case (2.6%). In patients with giant GIST compared with those with common GIST (diameter less than 10 cm), the proportion of mitotic figures, capsule rupture, multiple lesions, extra-gastrointestinal GIST, preoperative anemia and complications were significantly increased (all P<0.05). Of the 39 patients, 8 cases (20.5%) underwent simple tumor resection, 30 cases (76.9%) had synchronous partial resection of gastrointestinal and other organs, and tumor removal was unsuccessful in one case (2.6%); all cases were high risk GIST. A valid follow-up was conducted in 32 patients, of whom 4 cases (10.3%) had postoperative imatinib therapy; the 1- and 2-year progression-free survival rate was 92.8% and 79.6%, respectively. Conclusion: Giant GIST can be diagnosed according to clinical manifestations and results of imaging and pathological examinations, and a relatively high risk is suggested by its clinical features. Complete tumor excision combined with partial resection of gastrointestinal and other organs as well as standardized pre- and postoperative targeted therapy may be the effective approach to improve the outcome of these patients.