A clinical and pathological study of a case of retroperitoneal desmoplastic small round cell tumor
- VernacularTitle:腹膜后促结缔组织增生性小圆细胞瘤临床病理研究(附一例报告及文献复习)
- Author:
Ruijin ZHOU
;
Zhonghua LIU
;
Tongyu JI
- Publication Type:Journal Article
- Keywords:
Neoplasms;
Connective tissue;
Retroperitoneal neoplasms;
Pathology
- From:
Chinese Journal of Urology
1994;0(02):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinical and pathological features of a case of retroperitoneal desmoplastic small round cell tumor. Methods One case of desmoplastic small round cell tumor was presented with review of the literature. Results A 11 years old child complained of abdominal pain and a 9.0 cm?6.0 cm?3.0 cm cystic tumor located in the left retroperitoneal space near the left kindey was resected.The connective tissue was significantly proliferated around the tumor cell nests. Immunohistochemical staining revealed CD 99 (+) and EWS/WT1 (+). After resection of the tumor,chemotherapy was performed with DDP?ifosfamide and VP16. No tumor recorrence has been noted 16 months after operation. Conclusions Desmoplastic small round cell tumor has specific clinicopathologic stigmata occuring usually in young males and the tumor was characterized by expression of MIC2 gene product CD 99 and the EWS/WT1 protein. Surgical resection with cheomotherapy is the treatment of choice.
