Hepatoma in a child with neurofibromatosis

L. J. Ettinger and A. I. Freeman

A 6-year-old girl had neurofibromatosis and hepatocellular carcinoma (hepatoma). Although neurofibromatosis is a common disorder, hepatic cancers are rare in childhood. Hepatic cancer, Wilms' tumor, and adrenocortical neoplasia are all seen with increased frequency in association with certain congenital anomalies, particularly with hemihypertrophy and various hamartomas. Neurofibromatosis, a hamartomatous disorder, is also seen with these congenital anomalies and it is reported with an unusually high incidence in patients with Wilms' tumor. We therefore suggest that these disorders may be related.