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Soft-tissue tumors

Clear cell sarcoma

Soft tissueMalignant / sarcomaTumours of uncertain differentiation

Epidemiology

A rare sarcoma of young adults, often related to the tendons or aponeuroses of the limbs. Its relationship to tendons and aponeuroses and its occasional melanin can lead to confusion with metastatic melanoma.

Basic features

It can mimic a melanoma because of its melanocytic markers, but it is distinguished by its characteristic EWSR1-ATF1 fusion.

Usual treatment

The main treatment is wide resection with lymph node evaluation and additional therapies according to risk.

Outcomes and prognosis

The prognosis tends to be unfavorable in the long term: it responds poorly to chemotherapy and has a marked tendency toward late metastases, including to the lymph nodes, so it requires prolonged follow-up.

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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