musculoskeletal angiosarcoma

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Musculoskeletal angiosarcomas, (along with hemangiopericytomas and hemangioendotheliomas) are tumors 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.

Epidemiology

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

Pathology

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 hemangiomatous or lymphangiomatous cellular elements, which accounts for them previously being referred to as lymphangiosarcomas or haemangiosarcomas.

Radiographic features

Radiologically they appear quite similar to hemangiopericytomas and hemangioendotheliomas.

Plain radiograph

May be seen as a non-specific soft tissue mass. When involving bone, they are predominantly lytic, and may mimic hemangiomas 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 tumor from which vessels branch to encircle the tumor.

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 hemangiopericytomas.