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目的探讨胃肠道外间质瘤(EGIST)的临床表现、外科治疗及预后。方法回顾性分析2004年1月-2010年6月收治的35例EGIST患者的临床资料。男26例,女9例;年龄33～78岁,平均56岁。病程5 d～8个月,平均2个月。临床表现主要有腹部不适、腹痛及腹部包块等。均在术前行CT或腹部增强CT等检查发现病灶,其中位于系膜16例,网膜15例,腹膜后4例。35例均行手术治疗。结果术后均由病理学检查及免疫组织化学检测确诊,肿瘤标本镜下均以梭形细胞为主;极低危险、低危险、中危险、高危险患者分别为0、3、0、32例。免疫组织化学检测示酪氨酸激酶受体(CD117)、DOG-1、骨髓干细胞抗原(CD34)、酸性钙结合蛋白、平滑肌肌动蛋白、结蛋白阳性率分别为91.4%(32/35)、100%(3/3)、71.4%(25/35)、8.6%(3/35)、22.9%(8/35)、0%(0/35)。15例患者均获随访,时间19～96个月,平均46个月。8例出现进展,7例病情稳定。结论 EGIST发现时往往体积较大,预后较差,手术切除是首选治疗手段,甲磺酸伊马替尼对其具有较好的治疗效果。
Objective To investigate the clinical manifestation, surgical treatment and prognosis of extraintestinal stromal tumors (EGIST). Methods The clinical data of 35 patients with EGIST admitted from January 2004 to June 2010 were retrospectively analyzed. 26 males and 9 females; aged 33 to 78 years, mean 56 years. Duration of 5 d ~ 8 months, an average of 2 months. The main clinical manifestations of abdominal discomfort, abdominal pain and abdominal mass and so on. Preoperative CT or abdominal enhanced CT and other tests found lesions, including mesangial in 16 cases, 15 cases of retina, 4 cases of retroperitoneal. 35 patients underwent surgical treatment. Results All patients were diagnosed by pathological examination and immunohistochemistry after operation. Most of the tumor samples were spindle-shaped cells. The patients with very low risk, low risk, medium risk and high risk were 0, 3, 0 and 32 respectively . The positive rates of tyrosine kinase receptor (CD117), DOG-1, bone marrow stem cell antigen (CD34), acidic calcium-binding protein, smooth muscle actin and desmin were 91.4% (32/35) 100% (3/3), 71.4% (25/35), 8.6% (3/35), 22.9% (8/35), 0% (0/35). All 15 patients were followed up for 19 to 96 months with an average of 46 months. 8 cases of progress, 7 cases were stable. Conclusions EGIST is often found in large volume with poor prognosis. Surgical resection is the first choice of treatment and imatinib mesylate has a good therapeutic effect.