Familial intracranial ependymomas. Report of three cases in a family and review of the literature

Abstract

Familial cases of intracranial ependymomas have been well documented in the literature. The authors present two cases from a family in which three members harbored intracranial ependymomas. A 54-year-old man with fourth ventricular ependymoma underwent resection of the tumor followed by radiation therapy. His son presented at age 36 years with a fourth ventricular tanycytic ependymoma and underwent total resection of the ependymoma with postoperative radiation therapy. The father's sister had been treated at another institution for a posterior fossa ependymoma.The association of ependymomas with molecular genetic alterations in chromosome 22 has been previously described. Further investigation of the genetic influences may lead to better therapeutic approaches for this relatively rare clinicopathological entity.