Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Atypical Teratoid/Rhabdoid Tumor (AT/RT)

  • Jennifer TinkerEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_91


Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly malignant tumor of early childhood, most commonly diagnosed in infants who are less than 3 years. First described by Rorke and colleagues in 1987, the AT/RT received its designation because of its complex histological components. Prognosis is extremely poor with a median survival of 6–11 months. Factors associated with improved prognosis include supratentorial location, localized disease at the time of presentation, and complete resection (Torchia et al. 2015). Over half of AT/RTs identified are located within the posterior fossa (brainstem, cerebellum, and predominantly the cerebellopontine angle) (Rorke et al. 1996). Roughly one-fourth are supratentorial and 8% may be multifocal. Clinical presentation varies largely by tumor location and size. Infants, in particular, may present with nonspecific symptoms, including lethargy, vomiting, and failure to thrive. Older children (>3 years of age) may demonstrate more...

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References and Readings

  1. Lefkowitz, I. B., Rorke, L. B., & Packer, R. J. (1987). Atypical teratoid tumor of infancy: Definition of an entity. Annals of Neurology, 22, 56–65.Google Scholar
  2. Rorke, L. B., & Biegel, J. A. (2000). Atypical teratoid/rhabdoid tumour. In P. Kleihues & W. K. Cavenee (Eds.), World health organization classification of tumours. Pathology & genetics. Tumours of the nervous system (pp. 145–148). Lyons: IARC Press.Google Scholar
  3. Rorke, L. B., Packer, R. J., & Biegel, J. A. (1996). Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: Definition of an entity. Journal of Neurosurgery, 85, 56–65.PubMedCrossRefGoogle Scholar
  4. Torchia, J., et al. (2015). Molecular subgroups of atypical teratoid rhabdoid tumours in children: An integrated genomic and clinicopathological analysis. Lancet Oncology, 16(5), 569–582.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurologyThomas Jefferson UniversityPhiladelphiaUSA