A clinical and pathological study of a case of retroperitoneal desmoplastic small round cell tumor / 中华泌尿外科杂志

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.