Therapeutic targets of desmoplastic small round cell tumor and future direction
10.3969/j.issn.1000-8179.2020.13.540
- VernacularTitle: 促纤维增生性小圆细胞肿瘤靶向治疗的研究现状和展望
- Author:
Mingjin SHE
1
Author Information
1. Department of Oncology, Anhui Provincial Corps Chinese People's Armed Police Force
- Publication Type:Journal Article
- Keywords:
Desmoplastic small round cell tumor (DSRCT);
DNA damage repair;
EWSR1-WT1 fusion;
Immunotherapy;
Sarcoma;
Targeted therapy
- From:
Chinese Journal of Clinical Oncology
2020;47(13):695-699
- CountryChina
- Language:Chinese
-
Abstract:
Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant soft tissue sarcoma. Most patients are diagnosed at advanced stage unless DSRCT happens to be discovered accidentally. DSRCT mainly occurs in the abdomen and pelvis, spreading along the peritoneal surface. At the time of diagnosis, most patients have passed the operable stage. The diagnosis of DSRCT is based on the histological analysis of a biopsy. It typically manifests as small round blue cells in the nest, separated by a large number of fibroproliferative stroma. A stable cytogenetic feature of DSRCT is that the characteristic t(11; 22) (P13; Q12) chromosome produces the EWSWT1 fusion gene. The prognosis of patients with DSRCT is very poor, and the 5-year survival rate is about 15%. Despite the use of active treatment methods, such as chemotherapy, surgery, and total abdominal radiotherapy, about 60% 70% of patients with DSRCT die within 2- 3 years. Targeted therapy, immunotherapy, and other methods have been trialed recently in the treatment of DSRCT due to the development of DSRCT molecular genetics.
