PRIMARY INTRACRANIAL ATYPICAL TERATOID RHABDOID TUMOR IN A CHILD WITHCANAVAN DISEASE

A primary intracranial atypical teratoid/rhabdoid tumor was encountere d in a child (age 4 years and 9 months) with Canavan disease. The tumo r contained a large spindled cell component as well as classical rhabd oid morphology and focal areas resembling a primitive neuroectodermal tumor. The rhabdoid areas of the neoplasm were immunoreactive with ant ibodies against epithelial membrane antigen and vimentin, in the class ically described pattern. Ultrastructurally these portions of the tumo r displayed the characteristic perinuclear whorls of intermediate fila ments reported in rhabdoid tumors of all body sites. Thought to be pur ely coincidental, this is also the first description of any intracrani al neoplasm associated with Canavan disease.