Hepatic angiosarcoma in childhood. A case report and review of the literature.

We describe the clinical and pathologic features of an hepatic angiosarcoma in a 5-year-old child. The neoplasm manifested as a multicentric vascular tumor and was initially treated by lobectomy. Histopathologically, the lesion showed the features of infantile hemangioendothelioma, in which foci of cytologic atypia ("type 2") were present. A recurrence with rapid growth appeared that suggested a malignant tumor, but no metastases developed. A literature review confirmed the rarity of angiosarcomas in children and underscored the difficulties of formulating a prognostic estimate about infantile hemangioendotheliomas based on histology alone. Mitotic activity and cytologic atypism are regular features of angiosarcoma in children and cannot be ignored. This report also contrasts certain features of the biology of malignant vascular tumors of the liver in childhood with the counterpart in adults.