促纤维增生性小圆细胞瘤的治疗进展  

Research progresses of the treatment of desmoplastic small round cell tumor

作　　者：许霄 张凤春[1,3] 徐迎春[4] 

机构地区：[1]上海交通大学医学院附属苏州九龙医院肿瘤科,苏州215021 [2]广西医科大学第一附属医院放射科,南宁530021 [3]上海交通大学医学院附属瑞金医院肿瘤科,上海200025 [4]上海交通大学医学院附属仁济医院肿瘤科,上海200127

出　　处：《上海交通大学学报（医学版）》2016年第5期747-751,756,共6页Journal of Shanghai Jiao tong University:Medical Science

基　　金：国家自然科学基金(81301858)~~

摘　　要：促纤维增生性小圆细胞瘤(DSRCT)是一种罕见的、高度恶性、预后极差的恶性肿瘤,瘤细胞可起源于腹、盆腔腹膜及腹外器官,表现为腹腔内多发肉瘤样病变,多见于青少年及青年男性。病理组织学表现为纤维组织包绕的巢状分布的蓝色小圆细胞,共表达上皮性、神经源性及间质性标志,分子特征为t(11∶22)(p 13;q 12)染色体易位产生EWS-WT1融合蛋白。DSRCT的治疗尚无共识,主要包括减瘤性或根治性手术、高强度的多药联合化疗(新辅助、辅助)和全腹放疗。部分研究提示造血干细胞移植、靶向治疗、术后腹腔热化疗和术后调强适形放疗等可能提高生存期。该文就近年来DSRCT可选择的有循证医学证据支持的治疗措施作一综述。Desmoplastic small round cell tumor（ DSRC T） is a rare, highly malignant tumor with very poor prognosis. It often develops in the abdom inal and pelvic peritoneum or extra-abdom inal sites of adolescents and young m ales. Patients typically present with sym ptom s of abdom inal sarcom atosis. The histopathological m anifestation is featured with nest-distributed sm all round blue cells surrounded by fibrous tissue. This tum or can co-express epithelial, neural and mesenchym al markers. The molecular characteristic of D SRC Tis the production of EW S-W T1 fusion protein via the translocation of chrom osom e t（ 11： 22）（ p13; q12）. Standard management of D SRC Thas not been established so far. Treatm ents include debulking or radical surgery, high intensity adjuvant or neoadjuvant polychem otherapy, and whole abdom inal radiation. Som e studies suggested that m ethods such as hem atopoietic stem cell transplantation, targeted therapies, and postoperative hypertherm ic intraperitoneal chem otherapy（ H IPEC） and intensity-m odulated radiation therapy（ IM RT） can im prove survival. This paper review s recent evidence-base m edicine supported options for the treatm ent of D SRC T.

关 键 词：促纤维增生性小圆细胞瘤 诊断 治疗 

分 类 号：R738.6[医药卫生—肿瘤]