Dr. Francisco Bengoa
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Soft-tissue tumors

Dermatofibrosarcoma protuberans

Soft tissueIntermediate / locally aggressive or rarely metastasizingFibroblastic / myofibroblastic tumours

Epidemiology

An uncommon tumor of the skin and the tissue just beneath it, typical of young and middle-aged adults. It usually grows slowly in the skin and subcutaneous tissue, but it needs well-planned margins because its extensions can be wider than what is visible.

Basic features

A firm, slow-growing plaque or nodule that tends to invade the skin and subcutaneous tissue.

Usual treatment

Surgery with wide resection and clear (negative) margins. In cases that cannot be removed or that have spread, a targeted therapy (imatinib) is used, which acts on the tumor's characteristic genetic change (the COL1A1-PDGFB fusion).

Outcomes and prognosis

Good outlook with clear margins: recurrence is low after removal with wide margins, but high if the removal is incomplete. Metastasis is uncommon overall, but the risk rises meaningfully in the fibrosarcomatous variants.

Warning signs

These signs do not necessarily mean cancer, but they warrant evaluation before operating on or needling a mass.

  • A soft-tissue mass that grows, is larger than 5 cm, or is deep.
  • Persistent, night-time or progressive bone pain, especially if it limits activity.
  • A fracture from minimal trauma, or an aggressive bone lesion on an X-ray.
  • A lump that comes back after previous surgery.
  • Neurological symptoms, rapid swelling, or pain associated with a mass.

This information is general and does not replace a medical evaluation. A musculoskeletal tumor must be interpreted together with imaging, physical examination, biopsy when appropriate and each patient's context.

Specialist evaluation

If you have a mass, a bone lesion or a report suggesting a tumor, book an evaluation.

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