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Cerebellar primitive neuroectodermal tumor with multipotent differentiation in a family with von Hippel-Lindau
R Becker1, B L Bauer, H D Mennel
1Department of Neurosurgery, Philipps University Marburg, Germany.
Clinical Neuropathology
|March 1, 1993
Abstract:
A family with von Hippel-Lindau disease (vHLD) is presented. Three family members suffered from typical cerebellar hemangioblastomas. Another family member presented with a cerebellar neoplasm of different histology. Detailed histological analysis revealed a primitive neuroectodermal tumor (PNET) with neuronal, glial, myoid and ependymal differentiation. This is apparently the first description of the association of vHLD and a cerebellar PNET with multipotent differentiation.