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Soft-tissue tumors

Extraskeletal myxoid chondrosarcoma

Soft tissueMalignant / sarcomaTumours of uncertain differentiation

Epidemiology

A rare sarcoma of adults, usually in the deep tissues of the limbs. Despite the name, it does not arise in bone; it is usually deep and slow-growing, with possible late recurrences.

Basic features

It grows slowly and has a myxoid matrix; the name does not mean that it produces typical cartilage. It is defined by a rearrangement of the NR4A3 gene, which confirms the diagnosis against other myxoid-looking tumors.

Usual treatment

Wide resection is central; radiotherapy and systemic treatment are considered according to risk or advanced disease.

Outcomes and prognosis

It usually follows a prolonged course: the short-term prognosis is favorable, but it declines over the long term because of late recurrences and metastases.

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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