Angiosarcomas (like 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 therefore carrying an extremely poor prognosis), and often have local recurrences.
Epidemiology
They account for less than 1% of all sarcomas and 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 Stewart-Treves syndrome.
Pathology
The malignant cells express morphological and functional properties of endothelial cells. The tumours can be multicentric with haemorrhage and necrosis being common.
Location-specific lesions
- hepatic angiosarcoma 2
- breast angiosarcoma 5
- cutaneous angiosarcoma 3
- musculoskeletal angiosarcoma
- cardiac angiosarcoma
- primary angiosarcoma of spleen
- pulmonary angiosarcoma (can be primary or metastatic) 4