Angiosarcoma

Last revised by Mohammadtaghi Niknejad on 12 Jan 2024

Angiosarcomas (like 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 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 lymphedema 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 tumors can be multicentric with hemorrhage and necrosis being common.

Location-specific lesions
Associations

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