Atypical and polymorphic angioblastic tumors of the central nervous system

Five central nervous system (CNS) polymorphic angioblastic tumors have been studied. Four were located in the posterior fossa and one involved the temporal lobe. In reviewing the literature, the authors point out the rarity of the supratentorial locations and of malignant hemangioblastomas. The clinical and anatomopathological peculiarities are discussed, as is the uncertain classification of two cases. The discrepancy between histology and outcome is focused upon in the other three cases. Finally, the diagnostic, prognostic, and nosographic difficulties posed by CNS angioblastic neoplasms are discussed.