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 having local recurrences.
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 lymphedema which is clearly more common in women and is known as Stewart-Treves syndrome.
The malignant cells express morphological and functional properties of endothelial cells. The tumors can be multicentric with hemorrhage 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
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