Sugai Tamotsu, Uesugi Noriyuki, Yamada Noriyuki, Takahashi Keisuke, Wakabayashi Go, Terashima Masanori, Chiba Toshimi, Suzuki Kazuyuki
Division of Molecular Diagnostic Pathology, Dept. of Pathology, School of Medicine, Iwate Medical University.
Gan To Kagaku Ryoho. 2011 May;38(5):715-21.
Gastrointestinal stromal tumor (GIST)is a serious and predominantly sporadic tumor of the gastrointestinal tract (GIT). We attempted to review clinicopathological and molecular findings of GIST. GIST recently has been suggested to originate from the multipotential mesenchymal stem cells. Histologically, GIST is classified into spindle, epithelioid and mixed types. Tumor size, mitotic index, anatomic location and tumor rupture are the characteristics used to predict the clinical prognosis of patients. Expressions of c-kit/PDGFRA are essential for diagnosing GISTs. Activated mutant c-kit or platelet-derived growth factor receptor alpha (PDGFRA), which are potential therapeutic targets for imatinib, are constitutively expressed in most GIST. Imatinib selectively inhibits several protein tyrosine kinases and is an effective drug in malignant GISTs. More recently, sunitinib, a new KIT/PDGFRA kinase inhibitor, has been tested in patients with GIST resistant to imatinib with promising results.
胃肠道间质瘤（GIST）是一种严重的、主要为散发性的胃肠道（GIT）肿瘤。我们试图回顾胃肠道间质瘤的临床病理及分子学研究结果。最近有研究表明胃肠道间质瘤起源于多能间充质干细胞。从组织学上看，胃肠道间质瘤可分为梭形、上皮样和混合型。肿瘤大小、有丝分裂指数、解剖位置和肿瘤破裂情况是用于预测患者临床预后的特征。c-kit/PDGFRA的表达对于诊断胃肠道间质瘤至关重要。活化的突变型c-kit或血小板衍生生长因子受体α（PDGFRA）是伊马替尼潜在的治疗靶点，在大多数胃肠道间质瘤中呈组成性表达。伊马替尼选择性抑制多种蛋白酪氨酸激酶，是治疗恶性胃肠道间质瘤的有效药物。最近，一种新型KIT/PDGFRA激酶抑制剂舒尼替尼已在对伊马替尼耐药的胃肠道间质瘤患者中进行试验，结果令人满意。
