ABSTRACT

Epithelioid hemangioendotheliomas are unusual vascular neoplasms with biological behavior generally felt to be intermediate between malignant angiosarcomas and benign angiomas, although some workers now consider them to be fully malignant (Calonje and Fletcher, 2000). Since metastases occur in up to 30 percent of systemic examples, it is not surprising that case reports exist of epithelioid hemangioendotheliomas metastatic to the CNS (Palmieri et al., 2000). Hence, primary CNS epithelioid hemangioendothelioma must be distinguished from metastatic hemangioendothelioma, as well as metastases due to atrial myxomas (Samaratunga et al., 1994).