52例小肠间质瘤的临床、病理及影像学特征

Clinical,pathological and imaging characteristics of 52 cases of small intestinal stromal tumor

目的探讨小肠间质瘤（small intestinal stromal tumor,SIST）的临床、病理及影像学特征,为SIST的临床诊治提供经验。方法回顾性分析2010年3月-2014年3月武汉大学人民医院消化内科收治的经手术病理及免疫组化确诊的52例SIST患者的临床资料,总结其诊断及治疗的临床特征。结果术后6例患者出现消化道出血、伤口感染、电解质紊乱等并发症,经抗炎、止血及补液等对症支持治疗后均好转出院,无围手术期死亡病例。术后病理显示,肿瘤生长方式为腔内型或腔外型,组织形态均为小肠梭形细胞肿瘤;免疫组化显示,DOG-1及CD117阳性率最高。结合术后病理结果进行生物学危险度分级显示,中-低度危险性有20例,高度危险性有32例。42例中、低度危险性及部分高度危险性患者因个人原因术后未接受进一步药物治疗,未见肿瘤复发及转移。10例高度危险性患者术后给予口服甲磺酸伊马替尼IM辅助治疗,4例因病程较长、瘤体直径大、肿瘤出现破裂出血或坏死等原因于术后6~36个月出现复发转移;余6例患者随访未见肿瘤复发及转移。另22例高度危险性患者因经济原因仅行术后动态CT观察。结论 SIST临床上以消化道出血和腹痛为首发症状;双气囊小肠镜检查诊断是术前不可缺少的检查方法,如术前合理选择联合腹部CT小肠造影（CTE）或胶囊内镜,能够降低小肠镜操作难度,提供其发现率;DOG-1、CD117、CD34均为特异性病理标志物;外科手术切除为首选治疗方法,术后病理及免疫组化可对生物危险度进行分级,高度危险者应追加分子靶向治疗。

Objective To discuss the clinical,pathological,and imaging characteristics of small intestinal stromal tumor（ SIST）,so as to provide experience for the clinical diagnosis and treatment. Methods Fifty two cases of SIST in Department of Gastroenterology,Renmin Hospital of Wuhan University from Mar. 2010 to Mar. 2014 were analyzed retrospectively,all patients were confirmed by surgical pathology and immunohistochemistry,the clinical characteristics of diagnosis and treatment were summarized. Results After operation,6 cases appeared postoperative complications such as gastrointestinal hemorrhage,wound infection,electrolyte disturbance and so on,but after the supportive treatments of anti-inflammatory,hemostasis,fluid infusion,all the patients were recovered and discharged,there was no perioperative deaths. Postoperative pathology showed that the growth pattern of these tumors was intracavity type or extra-cavity type and all of them were small intestine spindle cell tumor on tissue morphology; Immunohistochemical studies displayed that the positive rates of DOG-1 and CD117 were the highest. Combined with postoperative pathologic results,biological risk assessment stratification indicated that there were 20 cases of low to medium risk and 32 cases of high risk. Forty two cases of low to medium risk and some high risk patients didn＇t accept further drug therapy after surgery for personal reasons,there was no tumor recurrence and metastasis. Ten cases of high risk SIST were given oral imatinib mesylate IM adjuvant therapy after surgery,among them,4 cases appeared postoperative recurrence or metastasis in 6 ~ 36 months due to long duration,large diameter tumor and tumor rupture hemorrhage or necrosis etc; the other 6 cases had no tumor recurrence and metastasis during follow-up. The other 22 cases of high risk SIST only did postoperative dynamic CT observation for economic reason. Conclusion Gastrointestinal bleeding and abdominal pain are the first symptoms of SIST; Double balloon endoscopy examination diagnosis is indispensable to pre-operation,if having reasonable choice before operation to combine with CT enterography（ CTE） or capsule endoscopy,it can reduce the operative difficulty of double balloon endoscopy and improve the discovery rate; DOG-1,CD34 and CD117 are all the specific immunohistochemical markers; surgical resection is the first choice of all treatments,postoperative pathology and immunohistochemistry can rank biological risk,the high risk paitients should receive additional molecular targeting treatment.