Abstract

The occurrence of hemangioblastoma in three brothers is reported. All of them developed posterior fossa tumors and one additionally developed a spinal tumor in the thoracic region. The clinical symptoms and signs manifested themselves in the early twenties in all three cases. None of the brothers demonstrated angiomatosis of the retina and only in one was mild erythrocytosis detected. Surgical removal was carried out in two patients and in one was limited only to a ventricular tap. In the remaining brother excision was followed by radiotherapy. Diagnosis was established on the basis of histologic examination in of the literature has shown that only in a few reports has the diagnosis been confirmed histologically in all presented cases. The importance of screening all the members of families in cases of suspected or diagnosed hemangioblastoma is emphasized.

Keywords: cerebellar hemangioblastoma, familial brain tumors, intramedullary spinal hemangioblastoma, posterior fossa tumors, recurrent brain tumors