dermatofibrosarcoma protuberans
MONDO:0011934Mondo
Findings
No curated finding names dermatofibrosarcoma protuberans yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22).
Definition from the Mondo Disease Ontology (MONDO:0011934), read 2026-09-29. CC BY 4.0.
Features
8 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.
- ErythemaHPOHP:0010783
- Very frequent (80% to 99% of cases)
- FibrosarcomaHPOHP:0100244
- Very frequent (80% to 99% of cases)
- Neoplasm of the skinHPOHP:0008069
- Very frequent (80% to 99% of cases)
- Subcutaneous noduleHPOHP:0001482
- Very frequent (80% to 99% of cases)
- Thickened skinHPOHP:0001072
- Very frequent (80% to 99% of cases)
- Skin ulcerHPOHP:0200042
- Frequent (30% to 79% of cases)
- PainHPOHP:0012531
- Occasional (5% to 29% of cases)
- LymphadenopathyHPOHP:0002716
- Very rare (1% to 4% of cases)
Where it sits
- Narrower terms (1)
Other names
2 names
Resolves to: dermatofibrosarcoma protuberans
- Also called
- dermatofibrosarcomaDFSP