Calabuig-Fariñas Silvia, López-Guerrero José Antonio, Navarro Samuel, Machado Isidro, Poveda Andrés, Pellín Antonio, Llombart-Bosch Antonio
University of Valencia, Valencia, Spain.
Int J Surg Pathol. 2011 Aug;19(4):448-61. doi: 10.1177/1066896911402327. Epub 2011 Mar 21.
Gastrointestinal stromal tumors (GISTs) are c-KIT-signaling-driven mesenchymal tumors of the human digestive tract, many of which have c-KIT or PDGFRα activating mutations. The authors studied the immunohistochemical markers, c-KIT and PDGFRα mutations, in GISTs and their association with the clinicopathological and clinical follow-up in 145 GISTs. Tumors were located mainly in the stomach, the median tumor size being 7.5 cm. The mitotic index was ≤5 mitoses per 50 high-power fields in 61% of cases, 96% expressed CD117, and c-KIT or PDGFRα mutations were detected in 68% of cases. The median follow-up of the series was 52 months (range = 1 to 244.9 months). Tumor size, cell morphology, mitotic index, incomplete resection, Fletcher's risk classification, Ki-67 overexpression, and c-KIT mutations were associated with progression-free survival. Incomplete resection and mitotic activity also provide information about overall survival. In conclusion, complete clinicopathological, immunohistochemical, and genetic descriptions are necessary to characterize this disease and optimize its clinical management.
胃肠道间质瘤(GISTs)是由c-KIT信号驱动的人类消化道间充质肿瘤,其中许多具有c-KIT或血小板衍生生长因子受体α(PDGFRα)激活突变。作者研究了145例GISTs中的免疫组化标志物、c-KIT和PDGFRα突变及其与临床病理和临床随访的相关性。肿瘤主要位于胃,肿瘤中位大小为7.5 cm。61%的病例有丝分裂指数≤每50个高倍视野5个有丝分裂,96%表达CD117,68%的病例检测到c-KIT或PDGFRα突变。该系列病例的中位随访时间为52个月(范围=1至244.9个月)。肿瘤大小、细胞形态、丝分裂指数、不完全切除、弗莱彻风险分类、Ki-67过表达和c-KIT突变与无进展生存期相关。不完全切除和有丝分裂活性也为总生存期提供信息。总之,完整的临床病理、免疫组化和基因描述对于表征该疾病和优化其临床管理是必要的。