Liposarcoma of hepatic hilum in childhood: report of a case and review of the literature.

Liposarcoma in childhood has seldom been documented. The rare occurrence of this tumor has resulted in a lack of information about its natural history, prognosis, and management. The differential diagnosis with lipoblastomatosis is obligatory. A 2 year 4 month-old white male child presented at the Hospital das Clinicas of Medical School of Ribeirão Preto, University of São Paulo, with fever, jaundice, and coluria secondary to a tumoral mass of the hepatic hilum causing obstruction of the biliary tree. Histologic and immunohistochemical study at autopsy disclosed a liposarcoma. A literature review found 64 other reported cases. Special attention was given to age, sex, anatomic localization, and histologic aspects of the literature cases. Liposarcoma involving the hepatic hilum and causing obstruction of the biliary tree in childhood was not previously reported in the literature.