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.
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Epidemiology
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.
Pathology
Location
They can occur in:
skin: 33%
soft tissues: 24%
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long bones: 60%
tibia: 23%
femur: 18%
humerus: 13%
pelvis: 7%
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Histology
Histologically they can contain either haemangiomatous or lymphangiomatous cellular elements, which accounts for them previously being referred to as lymphangiosarcomas or haemangiosarcomas.
Radiographic features
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 (DSA)
Dense, well-circumscribed areas of enhancement with early draining veins and shunting. Haemangiopericytomas 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.
See also
angiosarcoma (general article)
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