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 (DSRCT) is a rare, highly malignant tumor with very poor prognosis. It often develops in the abdominal and pelvic peritoneum or extra-abdominal sites of adolescents and young males. Patients typically present with symptoms of abdominal sarcomatosis. The histopathological manifestation is featured with nest-distributed small round blue cells surrounded by fibrous tissue. This tumor 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). Standard management of DSRCT has not been established so far. Treatments include debulking or radical surgery, high intensity adjuvant or neoadjuvant polychemotherapy, and whole abdominal radiation. Some studies suggested that methods such as hematopoietic stem cell transplantation, targeted therapies, and postoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and intensity-modulated radiation therapy (IMRT) can improve survival. This paper reviews recent evidence-base medicine supported options for the treatment of DSRCT.