Dr. Francisco Bengoa
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Soft-tissue tumors

Tenosynovial giant cell tumor

Soft tissueIntermediate / locally aggressive or rarely metastasizingFibrohistiocytic tumours

Epidemiology

It affects tendon sheaths or joints; the localized form is common in the hand, and the diffuse form in the knee or other joints. In joints it can present as recurrent effusions, locking, or stiffness, not only as a palpable mass.

Basic features

It causes swelling, stiffness, pain, or repeated joint effusions. The diffuse form was previously known as pigmented villonodular synovitis (PVNS).

Usual treatment

Removal of the tumor or of the joint lining (synovectomy). For the symptomatic diffuse form, when surgery is not possible or would mean a major loss of function, targeted systemic therapies against CSF1R (pexidartinib or vimseltinib) may be considered. These are specialist drugs and not a first-line option: pexidartinib requires strict monitoring of liver function because of the risk of serious liver injury, and the availability of both varies from country to country.

Outcomes and prognosis

The localized form is usually well controlled, with a low recurrence rate; the diffuse form recurs more often and requires long-term follow-up. Malignant transformation is exceptional.

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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