Advances of diagnosis and therapy for desmoplastic small round cell tumor
10.3760/cma.j.issn.1673-422X.2018.06.015
- VernacularTitle:促结缔组织增生性小圆细胞瘤诊疗进展
- Author:
Huoguang CHEN
1
;
Hongbiao WANG
;
Yingcheng LIN
Author Information
1. 515000,汕头大学医学院附属肿瘤医院肿瘤内科
- Keywords:
Diagnosis;
Therapy;
Desmoplastic small round cell tumor
- From:
Journal of International Oncology
2018;45(6):379-382
- CountryChina
- Language:Chinese
-
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 EWSWT1 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.
