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Atypical Teratoid Rhabdoid Tumor

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Abstract

Atypical teratoid/rhabdoid tumors are malignant high grade embryonal (WHO Grade IV) tumors seen in children often below the age of 3 years and rarely above the age of 6 years. Account for 10% of tumors in infants with slight male predominance, M:F ratio = 2:1 Characteristically poorly differentiated, contain rhabdoid cells with divergent differentiation to form epithelial, mesenchymal, neuronal, and glial components and may be associated with a subpopulation of primitive neuroectodermal cells.

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  • Rorke LB, Packer RJ, Biegel JA (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65

    Article  PubMed  CAS  Google Scholar 

  • Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL, Duffner PK, Kun LE, Perlman EJ (1998) Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma. Am J Surg Pathol 22:1083–1092

    Article  PubMed  CAS  Google Scholar 

  • Manhoff DT, Rorke LB, Yachnis AT (1995) Primary intracranial atypical teratoid/rhabdoid tumor in a child with Canavan disease. Pediatr Neurosurg 22:214–222

    Article  PubMed  CAS  Google Scholar 

  • Judkins AR, Mauger J, Rorke LB, Biegel JA (2004) Immunohistochemical analysis of hSNF5/INI 1 in pediatric CNS neoplasms. Am J Surg Pathol 28:644–650

    PubMed  Google Scholar 

  • Meyers SP, Khademian ZP, Biegel JA et al (2006) Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol 27:962–971

    PubMed  CAS  Google Scholar 

  • Giangaspero F, Rigobello L, Badiali M, Loda M, Andrieini L, Basso G, Zorzi F, Montaldi A (1992) Large cell medulloblastomas. A distinct variant with highly aggressive behavior. Am J Surg Pathol 16:687–693

    Article  PubMed  CAS  Google Scholar 

  • Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, Rosenblum MK, Yachnis A, Zhou H, Rorke LB, Biegel JA (2005) INI 1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol 64:391–397

    PubMed  CAS  Google Scholar 

  • Sévenet N, Sheridan E, Amram D, Schneider P, Handgretinger R, Delattre O (1999) Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers. Am J Hum Genet 65(5):1342–8

    Article  PubMed  Google Scholar 

  • Ammerlaan AC, Ararou A, Houben MP, Baas F, Tijssen CC, Teepen JL, Wesseling P, Hulsebos TJ (2008) Long-term survival and transmission of INI1-mutation via nonpenetrant males in a family with rhabdoid tumour predisposition syndrome. Br J Cancer 98(2):474–9

    Article  PubMed  CAS  Google Scholar 

  • Jackson EM, Shaikh TH, Gururangan S, Jones MC, Malkin D, Nikkel SM, Zuppan CW, Wainwright LM, Zhang F, Biegel JA (2007) High-density single nucleotide polymorphism array analysis in patients with germline deletions of 22q11.2 and malignant rhabdoid tumor. Hum Genet 122(2):117–27

    Article  PubMed  CAS  Google Scholar 

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Adesina, A.M., Hunter, J., Rorke-Adams, L. (2010). Atypical Teratoid Rhabdoid Tumor. In: Adesina, A., Tihan, T., Fuller, C., Poussaint, T. (eds) Atlas of Pediatric Brain Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-1062-2_8

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  • DOI: https://doi.org/10.1007/978-1-4419-1062-2_8

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-1061-5

  • Online ISBN: 978-1-4419-1062-2

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