胃肠道间质瘤治疗新进展

胃肠道间质瘤（gastrointestinal stromal tumor,GIST）是胃肠道最常见的间叶源性肿瘤.外科手术是局限性GIST患者的主要治疗手段,对于不能手术切除或复发转移的患者来说,对于传统的全身化疗和放疗不敏感性,使这部分患者的治疗相当棘手.约90%的GIST存在c-kit原癌基因获得性变异,使得酪氨酸激酶持续激活（无需配体即可使酪氨酸残基自体磷酸化）,导致细胞分裂异常、肿瘤增殖.伊马替尼选择性地抑制KIT相关的酪氨酸激酶的活性,使得伊马替尼治疗GIST.引起了广泛的关注.这里我们回顾了GIST治疗的最新进展和对于这些患者的最佳治疗.

New developments in gastrointestinal stromal tumor

Gastrointestinal stromal tumor(GIST) is the most common mesenchymal malignancy of the GI tract. The mainstay of treatment is surgery. For patients in whom complete resection is not possible, or in patients with metastatic or recurrent disease, they are unresponsive to standard chemotherapy and to radiotherapy. There has been no effective systemic treatment for unresectable GIST or metastatic disease. Gain-of-function mutations of the KIT proto-oncogene occur in up to 90% of GIST, allowing constitutive activation of tyrosine kinase (i.e. auto-phosphorylation of tyrosine residues independent of ligand-receptor binding), leading to aberrant cell division and tumour growth. Imatinib selectively inhibits the tyrosine kinase activity associated with KIT, which forms the rationale for evaluating its effects in GIST. Herein, we review recent developments in treatment for GIST and implication for optimal treatment in these patients.