A CASE OF familial polymorphous oligodendroglioma, occurring in a brot
her and sister, is presented. Polymorphous oligodendrogliomas have a c
haracteristic histopathology consisting of scattered multinucleated gi
ant cells against a typical oligodendroglial background. The oligodend
roglial character of the tumors was underlined by positive immunostain
ing for antigalactocerebroside, anticarbonic anhydrase, and anti-leu-7
, without expression of glial fibrillary acidic protein. Both tumors w
ere immunopositive for p53, suggesting a mutation in the p53 gene. No
incidence of cancer was recorded in the family. This is the first repo
rt of familial occurrence of this particular subtype of oligodendrogli
oma.