title: Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case.
abstract: Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.
authors.affiliation: Department of Neurology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, One children's Way, Little Rock, AR 72202, USA.
authors.forename: Bassem
authors.initials: B
authors.lastname: El-Nabbout
authors.forename: Rolla
authors.initials: R
authors.lastname: Shbarou
authors.forename: Charles M
authors.initials: CM
authors.lastname: Glasier
authors.forename: Ali G
authors.initials: AG
authors.lastname: Saad
mesh_terms: D016543:Central Nervous System Neoplasms / Q000473:pathology*; D002675:Child, Preschool; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008577:Meningeal Neoplasms / Q000473:pathology*; D018335:Rhabdoid Tumor / Q000473:pathology*