Dermatofibrosarcoma Protuberans
Also known as: DFSP
Rare slow-growing tumor of the dermis. Looks like a firm plaque or nodule on the trunk - low metastatic potential but high local recurrence.
What to look for
Side-by-side comparison
Normal skin
No firm plaques
Possible DFSP
Firm plaque, slowly growing
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma that begins in the dermis (middle layer of skin). It most commonly appears on the trunk (40-50% of cases), followed by the extremities and head/neck region.
DFSP typically starts as a firm, flesh-colored, reddish-brown, or violaceous plaque that slowly enlarges over months to years. It often grows as a raised, nodular mass that is attached to the skin but mobile over deeper tissues. Early DFSP may be mistaken for a scar, dermatofibroma, or keloid.
DFSP rarely metastasizes (less than 5% of cases) but has a notorious tendency for local recurrence after standard excision - rates of 20-50% with conventional surgery. Mohs micrographic surgery has dramatically reduced recurrence rates to under 5%.
A characteristic genetic feature of DFSP is a translocation between chromosomes 17 and 22, which produces the COL1A1-PDGFB fusion protein. This has led to the use of imatinib (a targeted therapy) for locally advanced or metastatic cases.
Quick self-check
Does this look like dermatofibrosarcoma protuberans? Answer 2 questions.
Is it a firm, slowly growing plaque or nodule on the trunk?
Has it been growing gradually over months or even years?
Risk factors
- Age 20-50 (most common in young to middle-aged adults)
- No clearly established environmental risk factors
- Previous trauma or surgical scars at the site (debated)
When to see a dermatologist
- ⚠A slowly growing, firm plaque or nodule on the trunk or limbs
- ⚠A raised skin growth that has been gradually enlarging for months
- ⚠A skin lump that feels attached to the skin but moves over underlying muscle
Often confused with
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Full ABCDE check →Frequently asked questions
Is DFSP dangerous?
DFSP rarely spreads to distant organs (under 5% of cases). The main concern is local recurrence - it has a tendency to grow back after standard surgery. Mohs surgery reduces recurrence to under 5%. Overall prognosis is good.
How is DFSP different from dermatofibroma?
Dermatofibroma is benign, typically small (under 1cm), and has a characteristic dimple sign when pinched. DFSP is larger, slowly growing, tends to be fixed to the skin, and does not dimple. Biopsy is needed to distinguish them definitively.