What Is the First-Line Treatment of Dermatofibrosarcoma Protuberans?

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive soft tissue tumour requiring careful evaluation of resectability before treatment selection. The first-line protocol addresses the full range of clinical situations: from disease amenable to complete surgical removal, through cases where margins are uncertain, to scenarios where resection is not feasible or would produce unacceptable functional or cosmetic results.

Treatment Approach

When technically achievable, surgical re-resection is the preferred initial step. For situations where adequate surgery is not possible, the protocol defines alternative strategies — including radiation therapy and, in specific molecularly confirmed cases, targeted systemic therapy. The precise criteria, sequencing, and conditions governing each option are set out in the full structured regimen.

Full algorithm — including molecular testing requirements and all eligibility criteria — available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

Re-resection if feasible (preferred)
RT if not given previously and resection is not feasible
Consider Imatinib in cases where disease is unresectable, or unacceptable functional or cosmetic outcomes will occur with resection

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