Intraventricular neuroblastoma in a patient wit von Hippel-Lindau's disease. Light and electron microscopic study.

Cerebral neuroblastoma is a rare neoplasm that has not previously been associated with a phakomatosis. We report a case of an intraventricular neuroblastoma in a patient with Von Hippel-Lindau's disease. The neoplasm consisted of process-forming cells containing clear vesicles, dense-cored granules and microtubules. An unusual finding was the presence of intertwining cytoplasmic processes, apparently representing aberrant process formation. We suggest that this case represents a cerebral neuroblastoma occurring as an unusual manifestation of a phakomatosis.