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Soft-tissue tumors

Spindle-cell neoplasm with NTRK fusion

Soft tissueMalignant / sarcomaTumours of uncertain differentiation

Epidemiology

An emerging molecular entity that can appear in children or adults, in the skin, subcutaneous tissue, or deep soft tissue. Identifying the NTRK fusion is clinically useful because targeted therapies exist for unresectable or advanced disease.

Basic features

It forms a morphological spectrum: at one end, low-grade infiltrative lesions (such as lipofibromatosis-like neural tumor); at the other, high-grade tumors. It usually presents as a soft-tissue mass, most often in the limbs.

Usual treatment

Resection is used for localized disease; if it is unresectable, recurrent, or metastatic, targeted therapy with TRK inhibitors (larotrectinib or entrectinib) is considered.

Outcomes and prognosis

Its behavior is variable: the spectrum ranges from low-grade lesions, with a mainly local risk, to high-grade sarcomas that can metastasize; so morphology, grade, and genetics are combined to plan treatment and 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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