Cerebellar primitive neuroectodermal tumor with multipotent differentiation in a family with von Hippel-Lindau disease. Case report.
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.
Department of Neurosurgery, Philipps University Marburg, Germany.
R
R
Becker
B L
BL
Bauer
H D
HD
Mennel
K H
KH
Plate
D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002528:Cerebellar Neoplasms / Q000235:genetics* / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006394:Hemangiosarcoma / Q000235:genetics / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008527:Medulloblastoma / Q000235:genetics / Q000473:pathology; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000473:pathology; D009373:Neoplasms, Germ Cell and Embryonal / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D006623:von Hippel-Lindau Disease / Q000235:genetics* / Q000473:pathology
D014408:Biomarkers, Tumor