促结缔组织增生性小圆细胞瘤诊疗进展

doi:10.3760/cma.j.issn.1673-422X.2018.06.015

摘要/Abstract

摘要： 促结缔组织增生性小圆细胞瘤（DSRCT）是一种高度恶性、预后极差的罕见恶性软组织肿瘤，主要发生于腹、盆腔，好发于青少年男性，易发生肝、肺等远处转移；病理组织学表现为小圆蓝细胞巢被促结缔组织间质包绕分隔，共表达上皮源性、神经源性及间质源性标志；分子特征为t（11；22）（p13；q12）染色体易位，EWSWT1基因融合。主要治疗手段包括根治性切除或最大限度减瘤术、高强度全身化疗、局部放疗及腹腔热灌注化疗。

关键词: 诊断, 治疗, 促结缔组织增生性小圆细胞瘤

Abstract: Desmoplastic small round cell tumor (DSRCT) is a rare and high malignant soft tissue tumor with very poor prognosis. It usually occurs in the abdominopelvic cavity of adolescents and young males. DSRCT is prone to occur distant metastasis, mainly in the liver and lung. The histopathological manifestation is featured with nests of small round blue cells separated by desmoplastic stroma. DSRCT can coexpress epithelial, neural and mesenchymal markers. The molecular characteristic of DSRCT is the production of EWS-WT1 fusion protein via the translocation of chromosome t (11; 22) (p13; q12). Treatments of DSRCT include radical resection or cytoreductive surgery, high intensity systemic chemotherapy, local radiotherapy and hyperthermic intraperitoneal chemotherapy.

Key words: Diagnosis, Therapy, Desmoplastic small round cell tumor

陈火光，王鸿彪，林英城. 促结缔组织增生性小圆细胞瘤诊疗进展[J]. 国际肿瘤学杂志, 2018, 45(6): 379-382.

Chen Huoguang, Wang Hongbiao, Lin Yingcheng. Advances of diagnosis and therapy for desmoplastic small round cell tumor[J]. Journal of International Oncology, 2018, 45(6): 379-382.

参考文献

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