Department of Surgery, University of Göttingen, Robert Koch Str. 42, 37075, Göttingen, Germany.
Int J Colorectal Dis. 2012 Jun;27(6):689-700. doi: 10.1007/s00384-011-1353-y. Epub 2011 Nov 29.
The gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the intestinal tract, known to be refractory to conventional chemotherapy or radiation. Its pathogenesis is defined by mutations within the KIT and PDGFRA gene, which constitutively activate KIT and PDGFRA oncoproteins, and serve as crucial diagnostic and therapeutic targets.
Besides surgery, therapy with imatinib mesylate, which inhibits KIT kinase activity, represents the other cornerstone for the treatment of GIST. Still, the only curative option for GIST is given after complete surgical removal even in a metastatic setting, but recurrence is common, and the risk can be defined by surgical factors like incomplete resection, intraperitoneal rupture, or bleeding and tumor associated factors like tumor size, mitotic index, or localization.
Consequently, adjuvant therapy with imatinib mesylate or other tyrosine kinase inhibitors is recommended for high-risk patients after complete resection. For unresectable and advanced GIST, a partial response or stable disease can be achieved in about 80% of patients with imatinib mesylate.
胃肠道间质瘤（GIST）是最常见的肠道间叶性肿瘤，对常规化疗或放疗具有抗药性。其发病机制是由 KIT 和 PDGFRA 基因突变定义的，这些突变会使 KIT 和 PDGFRA 癌蛋白持续激活，成为重要的诊断和治疗靶点。
除了手术外，抑制 KIT 激酶活性的甲磺酸伊马替尼治疗是 GIST 治疗的另一个基石。尽管如此，即使在转移环境下，完全手术切除仍然是 GIST 的唯一治愈选择，但复发很常见，风险可以通过手术因素如不完全切除、腹膜破裂或出血以及肿瘤相关因素如肿瘤大小、有丝分裂指数或定位来定义。
因此，对于完全切除后的高危患者，建议辅助使用甲磺酸伊马替尼或其他酪氨酸激酶抑制剂。对于不可切除和晚期 GIST，大约 80%的患者使用甲磺酸伊马替尼可获得部分缓解或疾病稳定。
