Lopez M, Comandone A, Adamo V, Apice G, Bearzi I, Bracci R, Carlini M, Carpano S, Condorelli S, Covello R, Cucchiara G, Di Filippo F, Doglietto G B, Ficorella C, Garofalo A, Gebbia N, Giuliani F, Massidda B, Messerini L, Palmirotta R, Tonelli F, Vidiri A
Istituto Nazionale Tumori "Regina Elena", Via Elio Chianesi, 53 - 00144 Roma, Italia.
Clin Ter. 2006 May-Jun;157(3):283-99.
Treatment of gastrointestinal stromal tumors (GIST) has been revolutioned by the recently discovered molecular mechanism responsible for the oncogenesis of this disease. In addition, due to the rapid progress at molecular and clinical level observed in the last few years, there is a need to review the current state of the art in order to delineate appropriate guidelines for the optimal management of these tumors. A panel of experts from several specialities, including medical oncology, surgery, pathology, molecular biology and imaging, were invited to participate in a meeting to present and discuss a number of pre-selected questions, and to achieve a consensus according to the categories of the National Comprehensive Cancer Network (NCCN) and the Standard Options Recommandations (SOR) of the French Federation of Cancer Centers. Generally, consensus points were from categories 2A of the NCCN and B2 of the SOR. Conventional histologic examination with immunohistochemistry for CD117, CD34, SMA, S-100 and desmin is considered standard. Molecular analysis for the identification of KIT and PDGFRA mutation may be indicated in CD117-negative GIST. Complete tumor resection with negative margins is the optimal surgical treatment. Adjuvant imatinib should be considered an experimental approach. Neoadjuvant imatinib is also experimental, although its use may be justified in unresectable or marginally resectable GIST. Imatinib should be started in metastatic or recurrent disease, and should be continued until progressive disease or drug intolerance. In these cases, sunitinib can be used. The optimal criteria for the assessment and monitoring of GIST undergoing imatinib therapy are not well known, but they should include reduction in tumor size and disease stabilization, as well as reduction of tumor density on CT scan and metabolic activity on PET scan.
胃肠道间质瘤（GIST）的治疗因最近发现的导致该疾病发生的分子机制而发生了变革。此外，由于在过去几年中分子和临床水平上取得的快速进展，有必要回顾当前的技术水平，以便为这些肿瘤的最佳管理制定适当的指南。来自包括医学肿瘤学、外科、病理学、分子生物学和影像学等多个专业的专家小组受邀参加一次会议，以提出并讨论一些预先选定的问题，并根据美国国立综合癌症网络（NCCN）的类别和法国癌症中心联合会的标准选项建议（SOR）达成共识。一般来说，共识点来自NCCN的2A类和SOR的B2类。采用针对CD117、CD34、平滑肌肌动蛋白（SMA）、S-100和结蛋白的免疫组织化学进行常规组织学检查被认为是标准方法。对于CD117阴性的GIST，可能需要进行分子分析以鉴定KIT和血小板衍生生长因子受体α（PDGFRA）突变。完整切除肿瘤且切缘阴性是最佳的手术治疗方法。辅助性伊马替尼应被视为一种实验性方法。新辅助性伊马替尼也是实验性的，尽管在不可切除或边缘可切除的GIST中使用可能是合理的。伊马替尼应在转移性或复发性疾病中开始使用，并应持续至疾病进展或出现药物不耐受。在这些情况下，可以使用舒尼替尼。对于接受伊马替尼治疗的GIST进行评估和监测的最佳标准尚不清楚，但应包括肿瘤大小缩小和疾病稳定，以及CT扫描上肿瘤密度降低和PET扫描上代谢活性降低。
