Gastrointestinal Stromal Tumor Advances in Diagnosis and Management
Gastrointestinal Stromal Tumor Advances in Diagnosis and Management
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DOI：
10.5858/arpa.2011-0022-ra
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发表时间：
2011-10-01
影响因子：
4.6
通讯作者：
Rubin, Brian P.
中科院分区：
文献类型：
--
作者：
Patil, Deepa T.;Rubin, Brian P.
背景：胃肠道间质瘤(GIST)是最常见的胃肠道间充质肿瘤，应与其他间充质肿瘤相鉴别。它们在试剂盒或血小板衍生生长因子受体a(PDGFRA)受体酪氨酸激酶中含有特定的激活突变，使它们对药物抑制剂如甲磺酸伊马替尼和苹果酸孙尼替尼有反应。目的：全面回顾GIST的发病机制和靶向治疗的基本原理，回顾有助于GIST与其他胃肠道间充质肿瘤区分的显著组织学和免疫组织化学特征，并提出指导临床治疗的风险分层的预后参数。资料来源：通过PubMed回顾英文文献以及个人经验。照片取自克利夫兰诊所遇到的病例。结论：KIT-GIST连接的发现不仅提高了GIST的诊断准确性，而且使我们对这些肿瘤的组织发生和分子发病机制有了更好的了解。(ARCH Pathol Lab Med.2011年；135：12981310；DOI：10.5858/arpa.2011-0022-RA)
Context.-Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract and should be differentiated from other mesenchymal tumors. They harbor specific activating mutations in the KIT or platelet-derived growth factor receptor a (PDGFRA) receptor tyrosine kinases, which makes them responsive to pharmacologic inhibitors, such as imatinib mesylate and sunitinib malate.Objectives.-To provide a comprehensive review of the pathogenesis of GIST and the underlying principles of targeted therapy, to review the salient histologic and immunohistochemical features that facilitate the distinction of GIST from other mesenchymal neoplasms of the gastrointestinal tract, and to present the prognostic parameters for risk stratification that guide clinical management.Data Sources.-Review of the English literature through PubMed as well as personal experience. Photographs were taken from cases encountered at the Cleveland Clinic.Conclusions.-The discovery of the KIT-GIST connection has not only improved the diagnostic accuracy of GISTs but also provided us with a better understanding of the histogenesis and molecular pathogenesis of these neoplasms. (Arch Pathol Lab Med. 2011;135:1298-1310; doi: 10.5858/arpa.2011-0022-RA)