Hepatic Neurofibromatosis, Malignant Schwannoma, and Angiosarcoma in von Recklinghausen's Disease

Liver involvement by neurofibromatosis is rare. This report describes a young man with von Recklinghausen's disease and hepatic neurofibromas who developed a large right hepatic lobe malignancy and died of massive intratumor hemorrhage. Postmortem examination showed the tumor to be composed of both malignant schwannoma and angiosarcoma and to have arisen from contiguous neurofibromas in portal tracts. Widespread pulmonary metastases consisted of the angiosarcomatous elements alone. The expression of malignant schwannoma and angiosarcoma phenotypes in this tumor may be related to a common histogenesis from cells of the neural crest.