Primary Hepatic Infantile Hemangioendothelioma.

Abstract

Primary hepatic infantile hemangioendothelioma is a rare tumor, characterized by its appearance in early infancy as well as by the frequent association with various malformations and with similar angiomatous tumors of other tissues and organs. It may be single or multinodular and it is often complicated by severe peritoneal hemorrage. Two histologic types of this tumor are known: tipe 1 is sharply circumscribed and is considered similar to so called capillary hypertrophic hemangioma, while type 2, by showing aggressive, infiltrative and anaplastic features, is rather close to angiosarcoma. Clinical and pathological data concerning two cases of type 2 primary hepatic infantile hemangioendothelioma are reported. On the basis of experimental results and epidemiological data on hepatic angiosarcoma of adults, a possible etiopathogenetic relationship with maternal exposure during pregnancy to dangerous environmental factors is postulated.