CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system

Introduction Atypical teratoid/rhabdoid tumors (ATRT) are rare aggressive neoplasms of the CNS affecting predominantly very young children. Methods We retrospectively reviewed the imaging findings of 9 CT and 32 MR examinations of the brain and spine of 33 children. Results Of the 33 tumors, 11 were located in the infratentorial compartment, 16 in the supratentorial compartment, 5 in both cranial compartments, and 1 in the lower thoracic spinal cord. The mean age of the children with infratentorial or infra- and supratentorial tumors was significantly lower than the mean age of the children with purely supratentorial tumors. Heterogeneity on imaging, large size and high tumor stages are striking features reflecting the aggressive nature of this histopathological entity. Although not present in the majority of children, a distinct and unusual pattern of a wavy band-like enhancement surrounding a central hypointensity was present in 12 of 32 children (38%) in whom contrast medium was used. Conclusion To the best of our knowledge this is the largest number of imaging examinations of ATRTs so far reported. A rather unusual pattern of contrast enhancement may be typical of ATRTs.