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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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.
Sources
Based on clinical guidelines from the American Academy of Dermatology (AAD) and British Association of Dermatologists (BAD). Statistics from NCI SEER Program and IARC GLOBOCAN. Staging per AJCC Cancer Staging Manual, 8th ed. Full methodology