Dr. Francisco Bengoa
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Soft-tissue tumors

Sclerosing epithelioid fibrosarcoma

Soft tissueMalignant / sarcomaFibroblastic / myofibroblastic tumours

Epidemiology

A rare sarcoma of adults, usually located deep in the limbs, trunk, or pelvic girdle. It is a rare sarcoma that can share features with other fibromyxoid tumors; molecular tests help confirm it.

Basic features

It has a dense collagenous matrix and can be mistaken for less aggressive lesions if the biopsy is limited. MUC4 expression and the EWSR1::CREB3L1 fusion are the tests that confirm it and separate it from other fibromyxoid lesions.

Usual treatment

It requires wide resection and discussion of radiotherapy or systemic therapy in a specialized board.

Outcomes and prognosis

It can recur and spread late; long-term follow-up is essential.

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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