Dr. Francisco Bengoa
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Soft-tissue tumors

Alveolar rhabdomyosarcoma

Soft tissueMalignant / sarcomaSkeletal muscle tumours

Epidemiology

A subtype that is relatively more common in adolescents and young adults, and occurs more often in the limbs or trunk than the embryonal subtype (the most common one in childhood). FOXO1 fusions, when present, change risk stratification and follow-up.

Basic features

It is usually associated with the PAX3/PAX7-FOXO1 fusions and behaves more aggressively than the embryonal subtype; cases without that fusion (fusion-negative) behave like the embryonal subtype.

Usual treatment

It requires multimodal chemotherapy and local control with surgery, radiotherapy, or both. Before chemotherapy starts it is worth discussing fertility preservation with the team, because some of these treatments can affect it and the options have to be raised beforehand.

Outcomes and prognosis

It usually has a worse prognosis than the embryonal subtype: the PAX-FOXO1 fusion, metastases, and the initial response strongly influence outcome, with a more guarded prognosis in higher-risk disease.

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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