Dr. Francisco Bengoa
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Soft-tissue tumors

Malignant peripheral nerve sheath tumor

Soft tissueMalignant / sarcomaPeripheral nerve sheath tumours

Epidemiology

A rare sarcoma; the risk increases in neurofibromatosis type 1 and after prior radiotherapy. In neurofibromatosis type 1, rapid growth, new pain, or a neurological deficit in a neurofibroma are warning signs.

Basic features

A painful or rapidly growing mass along the course of a nerve, with a possible neurological deficit.

Usual treatment

Wide resection when possible, radiotherapy, and systemic treatment depending on grade, size, and extent.

Outcomes and prognosis

The prognosis is guarded and tends to be worse in the setting of neurofibromatosis type 1. It has a higher risk of recurrence and metastasis than benign nerve tumors and requires specialized 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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