desmoplastic small round cell tumor
Findings
No curated finding names desmoplastic small round cell tumor yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0019373), read 2026-09-29. CC BY 4.0.
Features
18 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abdominal distentionHPOHP:0003270
- Very frequent (80% to 99% of cases)
- Abdominal painHPOHP:0002027
- Very frequent (80% to 99% of cases)
- Abnormal peritoneum morphologyHPOHP:0002585
- Very frequent (80% to 99% of cases)
- LymphadenopathyHPOHP:0002716
- Very frequent (80% to 99% of cases)
- SarcomaHPOHP:0100242
- Very frequent (80% to 99% of cases)
- HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
- IleusHPO
Show the remaining 6
- CachexiaHPOHP:0004326
- Occasional (5% to 29% of cases)
- Neoplasm of the central nervous systemHPOHP:0100006
- Occasional (5% to 29% of cases)
- Neoplasm of the lungHPOHP:0100526
- Occasional (5% to 29% of cases)
- Neoplasm of the pancreasHPOHP:0002894
- Occasional (5% to 29% of cases)
- Ovarian neoplasmHPOHP:0100615
- Occasional (5% to 29% of cases)
- Testicular neoplasmHPOHP:0010788
- Occasional (5% to 29% of cases)
Where it sits
Other names
9 names
Resolves to: desmoplastic small round cell tumor
- Also called
- Desmoplas. small round cell tumorDesmoplas. small round cell tumourDesmoplastic small round cell tumourDesmoplastic small round-cell neoplasmDesmoplastic small round-cell tumorDesmoplastic small round-cell tumourDSRCTPolyphenotypic small round cell tumorPolyphenotypic small round cell tumour