HEPATIC HEMANGIOENDOTHELIOMAS, PLACENTAL CHORIOANGIOMAS, AND DYSMORPHIC KIDNEYS IN BECKWITH-WIEDEMANN SYNDROME

A 4-month-old female, birth weight 3150 g, had a history of maternal eclampsia, multiple placental chorioangiomas, and persistent neonatal hypoglycemia. Macroglossia and enlarged kidneys were recorded. Autopsy revealed multiple hepatic hemangioendotheliomas (type 1), massive cardiomegaly, and bilateral nephromegaly. Both kidneys were lobulated with active glomerulogenesis and clusters of immature tubules and foci of dysplastic medullary ducts. The features suggest that the nephrogenesis was secondary to the persistence of actively branching nephron-inducing ducts. Nodular hyperplasia of the adrenal cortex (adrenoblastomatosis) was present. This report expands the list of tumors to be found in Beckwith-Wiedemann syndrome (BWS).