Dermatofibrosarcoma (DFSP) is a rare, locally aggressive skin tumour. It appears in the form of indurated plaques or nodules of flesh colour and progressive growth, mainly on the trunk and lower limbs. Diagnosis is based on anatomopathological analysis, with immunostaining to rule out fibrosarcomatous transformation, which occurs in 10-15% of cases. Treatment is based on surgery, with two options: Mohs micrographic surgery when available, to reduce recurrence, or wide excision with 2-3cm margins. In the case of inoperable or metastatic DFSP, imatinib may be proposed. Recurrence occurs with a median delay of 4 to 5 years, justifying regular follow-up.
Keywords: A rare skin tumour; Dermatofibrosarcoma; Dermatofibrosarcome; Tumeur cutanée rare.
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