/desmoplastic small round cell tumor

desmoplastic small round cell tumor

Rare Disease

MONDO:0019373

Also Known As

DSRCTDesmoplas. small round cell tumorDesmoplas. small round cell tumourDesmoplastic small round cell tumorDesmoplastic small round cell tumourDesmoplastic small round-cell neoplasmDesmoplastic small round-cell tumorDesmoplastic small round-cell tumourPolyphenotypic small round cell tumorPolyphenotypic small round cell tumourdesmoplastic small round cell tumordesmoplastic small-round-cell tumordesmoplastic small-round-cell tumour

Definition

Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases.