Hepatic hemangioendothelioma: successful treatment with steroid in a very-low-birth-weight infant

A female infant, born at the gestational age of 29 weeks with a birthweight of 1,350 gm, developed progressive hepatomegaly at 10 days of age. Congestive heart failure gradually developed, and hepatic hemangioendothelioma was diagnosed at 1 month of age by open biopsy. Due to rapid enlargement of the tumor and progressive heart failure, steroid therapy was given from 36 days of age, including methylprednisolone 15 mg/kg/day for 3 days and 10 mg/kg/day for 4 days, then prednisolone 4 mg/kg/day for 20 days followed by tapering till 74 days of age. The tumor regressed gradually and was not detectable by sonography at 33 months of age. We suggest that, even in very-low-birthweight infants, the prognosis of hepatic hemangioendothelioma may be improved with aggressive therapy when symptoms develop during tumor progression, and that steroid should be the initial treatment.