Dr. Francisco Bengoa
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Bone tumors

Epithelioid hemangioendothelioma of bone

BoneMalignant / sarcomaVascular tumours of bone

Epidemiology

A low- to intermediate-grade malignant vascular tumor that can be multifocal in bone. Its behavior is variable; the extent in bone and soft tissue determines whether surgery, radiotherapy, or systemic therapy predominates.

Basic features

It often causes pain and multiple lytic (bone-thinning) lesions and can be mistaken for metastasis or myeloma. The diagnosis is confirmed by the characteristic WWTR1-CAMTA1 fusion (or, less often, YAP1-TFE3), which avoids treating it as a metastasis from an unknown primary.

Usual treatment

Treatment combines local control, a systemic evaluation, and cancer therapies according to the extent of disease and which organs are involved.

Outcomes and prognosis

The course varies: some forms are indolent (slow-growing) while others progress, so it requires expert 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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