18例直肠间质瘤的诊治分析

The Diagnosis and Treatment of 18 Cases of Rectal Gastrointestinal Stromal Tumors

目的:直肠间质瘤(Gastrointestinal stromal tumour,GIST)是相对少见的疾病,缺少统一的治疗规范。本研究探讨直肠间质瘤的临床特点、诊断及治疗方法。方法:对中南大学湘雅医院2002年1月～2009年4月间收治的18例直肠GIST的临床资料、治疗方法及结果进行回顾性分析。结果:直肠GIST的临床表现无特异性,多表现为血便或大便次数增多。CT或MRI显示肿块边界清楚,瘤内有出血坏死及无淋巴结肿大。18例标本免疫组化检测CD117和CD34阳性率均100%。低危险性和极低危险性共8例,占44.4%。全组均经手术治疗,行局部切除12例,直肠前切除术(Dixon)3例,腹会阴联合切除术(Miles)3例。术前以伊马替尼新辅助化疗3例,均达部分缓解(partial response,PR),然后均行局部切除。随访1～84个月,5例患者复发转移,其中3例行伊马替尼治疗,病情稳定。另外2例未接受伊马替尼治疗,死亡1例,另1例反复局部切除后行Miles手术。局部切除组与Miles组的无复发生存时间分别为(75.0±8.4)个月和(26.0±11.1)个月(P=0.023)。结论:直肠GIST的治疗方法应有别于直肠癌,需要制定个体化的外科治疗方案。对大部分低危险性的患者可通过各种手术路径行局部切除而达到满意效果。伊马替尼新辅助化疗的应用,可使部分直肠GIST患者获得保肛机会。

Objective： To assess the clinical features, diagnosis and treatment of gastrointestinal stromal tumor in the rectum.Methods： Records of 18 patients diagnosed as GIST in the rectum between January 2002 and April 2009 were re-viewed and the major clinical features, treatment modalities and outcomes were analyzed.Results： The clinical features of GIST in the rectum were nonspecific.Most patients manifested with bloody stool or changes in bowel habits.CT scan or MRI findings showed necrosis and/or hemorrhage in the tumor and well defined tumor margins.Even in the case of large GIST, no lymphadenopathy was not found, which could be a factor for the differential diagnosis of GIST from other rectal neoplasms.All of the resected tumor specimens showed positive expression of CD117 and CD34 in immunohistochemical staining.Low and very low risk patients accounted for 44.4% （8/18）.All patients received surgery.Twelve patients were treated with local excision with different approaches.Anterior resection of the rectum （Dixon） was undertaken in three pa-tients and abdominoperineal resection （Miles） in three patients.Neoadjuvant therapy with imatinib was applied for three pa-tients with partial response.After a median follow-up of 34 months （1～84 months）, recurrence and/or metastasis occurred in five patients, and three of them were treated with imatinib.One patient received Miles surgery after repeated local exci-sions.Only one patient died of bone metastasis.Recurrence-free survival （RFS） of the local excision group was longer than that of abdominoperineal resection （APR） group （75.0±8.4 months vs 26.0±11.1 months, P=0.023）.Conclusion： The treatment for rectal GIST should be individualized and be different from that of rectal cancer.Treatment decision and choice of procedures should be based on careful preoperative evaluation of tumor size, location, extent and risk level.Most of the anorectal GIST were rated as low-risk in this cohort and could be excised locally by different approaches with satisfactory outcome.Neoadjuvant therapy with imatinib may benefit some patients to obtain the opportunity of sphincter-saving.