Atypical teratoid and rhabdoid tumor. Report of a congenital case

The atypical teratoid rhabdoid tumor is a rare brain tumor of childhood. We report a congenital case, revealed by peripheral facial palsy. This polymo rphous tumor consisted of rhabdoid cells associated with areas of primitive neuroectodermal tumor. The immunoreactivity for the three proteins vimenti n, epithelial membrane antigen and smooth-muscle actin was suggestive of rh abdoid tumor. Genetic study showed a homozygous mutation in the tumoral DNA and a constitutional heterozygous mutation, as it was demonstrated in atyp ical teratoid rhabdoid tumors and in renal and extra-renal rhabdoid tumors.