Familial occurrence of polymorphous oligodendroglioma.
A case of familial polymorphous oligodendroglioma, occurring in a brother and sister, is presented. Polymorphous oligodendrogliomas have a characteristic histopathology consisting of scattered multinucleated giant cells against a typical oligodendroglial background. The oligodendroglial character of the tumors was underlined by positive immunostaining for antigalactocerebroside, anticarbonic anhydrase, and anti-leu-7, without expression of glial fibrillary acidic protein. Both tumors were immunopositive for p53, suggesting a mutation in the p53 gene. No incidence of cancer was recorded in the family. This is the first report of familial occurrence of this particular subtype of oligodendroglioma.
Kros
J M
JM
Department of Pathology,/Neuropathology, University Hospital Rotterdam-Dijkzigt, The Netherlands.
Lie
S T
ST
Stefanko
S Z
SZ
D000328:Adult; D001932:Brain Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D002648:Child; D017809:Fatal Outcome; D005260:Female; D005625:Frontal Lobe / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D009837:Oligodendroglioma / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D010296:Parietal Lobe / Q000473:pathology / Q000601:surgery; D013702:Temporal Lobe / Q000473:pathology / Q000601:surgery