Musculoskeletal angiosarcoma

Musculoskeletal angiosarcomas, (along with haemangiopericytomas and haemangioendotheliomas) are tumours that arise from vascular structures. They are typically difficult to distinguish from one another on imaging alone.

Angiosarcomas, are the most aggressive of the three, frequently having metastases at the time of diagnosis, and often having local recurrences. 

They are more frequently seen in males (M:F 2:1) except when seen in the setting of post-mastectomy lymphoedema which is clearly more common in women and is known as the Stewart-Treves syndrome.

Location

They can occur in:

  • skin: 33%
  • soft tissues: 24%
  • angiosarcoma of bone: 6%
    • long bones: 60%
      • tibia: 23%
      • femur: 18%
      • humerus: 13%
      • pelvis: 7%
Histology

Histologically they can contain either haemangiomatous or lymphangiomatous cellular elements, which accounts for them previously being referred to as lymphangiosarcomas or haemangiosarcomas.

Radiologically they appear quite similar to haemangiopericytomas and haemangioendotheliomas.

Plain radiograph

May be seen as a non-specific soft tissue mass. When involving bone, they are predominantly lytic, and may mimic haemangiomas with a course honeycomb appearance.

Angiography

Dense, well-circumscribed areas of enhancement with early draining veins and shunting. Hemangiopericytomas are described as having a pedicle formed by the arteries supplying the tumour from which vessels branch to encircle the tumour.

CT

Non-specific soft tissue mass, with a density similar to muscle but demonstrating bright contrast enhancement. CTA may demonstrate large feeding vessels.

MRI

Brightly enhancing soft tissue mass, often hyperintense on T2WI, with prominent flow voids, most marked in haemangiopericytomas.

Bone tumours

The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the paediatric patient.

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Article Information

rID: 901
Section: Pathology
Synonyms or Alternate Spellings:
  • Angiosarcoma (MSK)
  • Angiosarcoma - musculoskeletal

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