Dr. Francisco Bengoa
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Soft-tissue tumors

Epithelioid hemangioendothelioma of soft tissue

Soft tissueMalignant / sarcomaVascular tumours

Epidemiology

A low- to intermediate-grade malignant vascular neoplasm that can affect soft tissue or bone. It can be multifocal within the musculoskeletal system; the initial extent guides surveillance and treatment.

Basic features

It can be multifocal and follow an unpredictable course, with pain or a mass depending on the site. It is confirmed by the characteristic WWTR1-CAMTA1 fusion (or YAP1-TFE3 in a minority), a key finding because its epithelioid appearance can be mistaken for a metastatic carcinoma.

Usual treatment

Management ranges from observation in selected cases to surgery, radiotherapy, or systemic treatment depending on the extent and symptoms.

Outcomes and prognosis

The course may be slow or progressive; the prognosis is variable — often intermediate-favorable, but worse with extensive disease. The size, symptoms, and extent guide 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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