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.
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Kawaguchi T et al (2004) Atypical teratoid/rhabdoid tumour with leptomeningeal dissemination in an adult. Acta Neurochir (Wien) 146(9):1033–1038 discussion 1038
Burger PC et al (1998) Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 22(9):1083–1092
Bailey P, Robitaille Y (1985) Primary diffuse leptomeningeal gliomatosis. Can J Neurol Sci 12(3):278–281
Dirks PB et al (1996) Supratentorial primitive neuroectodermal tumors in children. J Neurooncol 29(1):75–84
Wada C et al (1986) Primary leptomeningeal ependymoblastoma. Case report. J Neurosurg 64(6):968–973
Rogers LR et al (1995) Primary leptomeningeal oligodendroglioma: case report. Neurosurgery 36(1):166–168 discussion 169
Kitahara M et al (1985) Diffuse form of primary leptomeningeal gliomatosis. Case report. J Neurosurg 63(2):283–287
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El-Nabbout, B., Shbarou, R., Glasier, C.M. et al. Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case. J Neurooncol 98, 431–434 (2010). https://doi.org/10.1007/s11060-009-0094-z
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DOI: https://doi.org/10.1007/s11060-009-0094-z