小肠胃肠道间质瘤的诊断与治疗

Diagnosis and treatment for gastrointestinal tract stromal tumors of small intestine

目的探讨小肠胃肠道间质瘤(GIST)的诊断、综合治疗疗效及预后。方法回顾性分析GIST25例临床资料。结果恶性GIST23例(92.0%),交界性恶性GIST2例(8.0%)。临床常见的症状为腹痛、腹部肿块、消化道出血。肿瘤完全切除23例(92.0%),姑息性切除2例。免疫组织化学检测CD117,CD34阳性率分别为94.4%(17/18)和50.0%(10/20)。本组无手术死亡病例。平均随访时间8年,复发和转移18例。全部病例术后口服伊玛替尼(格列卫),中位无瘤生存期短期服药组(15±3)月,长期服药组(52±3)月。全组无瘤生存期(35±13)月,转移、复发后中位生存期未治疗组(6±0.3)月,单纯服药组(23±10)月;服药加手术组(51±11)月,平均生存期(12±13)月。短期服药组与长期服药组1、3、5年无瘤生存率分别为57.0%、21.0%、21.0%和100.0%、82.0%、36.0%。肿瘤转移、复发后未治疗组,单纯服药组与服药手术组0.5、1、3、5年肿瘤复发后生存率分别为14.0%、0.0%、0.0%、0.0%,64.0%、51.0%、0.0%、0.0%和88.0%、83.0%、76.0%、21.0%。结论小肠GIST临床症状缺乏特异性,确诊依靠临床及病理,免疫组织化学染色CD117、CD34阳性的联合诊断。肿瘤的完整切除和分子靶向综合治疗是小肠GIST的主要手段。肿瘤复发转移后的积极治疗是获得良好预后的重要途径。

Objective To review the diagnosis,treatment and prognosis of gastrointestinal stromal tumors（GIST） of the small intestine.Methods The clinicopathological and follow-up data of 25 patients with GIST of the small intestine admitted to Cancer Hospital during March 1999 to May 2006 were analyzed retrospectively.Results The pathological examination showed malignant lesions in 23 cases（92.0%）,junctional lesions in 2 cases（8.0%）.The clinical signs included gastrointestinal bleeding,epigastric pain and mass in abdomen.Twenty-five patients underwent operation,including radical operations in 23 cases and palliative operations in 2 cases.The positive rate of CDll7 was 94.4%（17/18） and that of CD34 was 50.0%（10/20）.There was no surgical mortality.The average follow-up time was 8 years and 18 cases had recurrence of metastasis.All cases received therapy of imatinib after operation.The median period of disease-free survival（DFS） in patients with short-term imatinib therapy was（15±3）months;that with long-term therapy was（52±3）months;the overall DFS time was（35±13）months.The median survival period for cases of recurrence and metastasis with no therapy was（6±0.3）months;that for cases with imatinib therapy was（23±10）months;for cases with imatinib therapy plus operation was（51±11）months;the overall median survival period of the cases with recurrence and metastasis was（12±13）months.The 1-,3-,5-year median survival rates of DFS with short-term and long-term of imatinib therapy were 57.0%,21.0%,21.0% and 100.0%,82.0%,36.0%,respectively.The 0.5-,1-,3-,5-year median survival rates for cases of recurrence and metastasis with no therapy,imatinib therapy and imatinib therapy plus operation were 14.0%,0.0%,0.0%,0.0%;64.0%,51.0%,0.0%,0.0% and 88.0%,83.0%,76.0%,21.0%,respectively.Conclusions The diagnosis of GIST of small intestine relies on clinical,histopathological and immunohistochemical examinations because of lacking specific clinical manifestations.CD117 and CD34 are important markers for the diagnosis of GIST of small intestine.The curative-intent operation and molecular target therapy are important in treatment of GIST.The active therapy including the molecular target therapy and reoperation will prolong the survival of patients with GIST of small intestine who suffered from postoperative recurrence and metastasis.