Skip to main content
SpringerLink
Log in

Pediatric Atypical Teratoid/Rhabdoid Tumors (An Overview)

  • Chapter
  • First Online:
Pediatric Cancer, Volume 2

Part of the book series: Pediatric Cancer ((PECA,volume 2))

  • 1396 Accesses

Abstract

Pediatric Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system (CNS) are among the most malignant neoplasms and most often diagnosed in children smaller than 3 years of age and incidence is 1–2% of all brain tumors in children. Sixty-three percent of the AT/RT of the CNS is seen in infra-tentorial compartment, there are no precise imaging features that differentiate AT/RT from the other posterior fossa tumor. The “rhabdoid” cells are characteristic on cytopathology. It has been established now that CNS, AT/RT often shows deletion of the long arm of chromosome 22q11.2. The initial treatment for most children with AT/RT is surgical with and without cerebrospinal fluid diversionary procedure. Children with less than 3 years of age offered chemotherapy but in older children radiotherapy is given in addition.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P (1996) Effects of medulloblastoma resections on outcome in children: a report from the Children’s Cancer Group. Neurosurgery 38:265–271

    Article  PubMed  CAS  Google Scholar 

  • Bambakidis NC, Robinson S, Cohen M, Cohen AR (2002) Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features. Pediatr Neurosurg 37:64–70

    Article  PubMed  Google Scholar 

  • Beckwith JB, Palmer NF (1978) Histopathology and prognosis of Wilm’s tumors: results from the First National Wilm’s Tumor Study. Cancer 41:1937–1948

    Article  PubMed  CAS  Google Scholar 

  • Bhattacharjee M, Hicks J, Langford L, Dauser R, Strother D, Chintagumpala M (1997) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. Ultrastruct Pathol 21:369–378

    Article  PubMed  CAS  Google Scholar 

  • Biegel JA (1997) Genetics of pediatric central nervous system tumors. J Pediatr Hematol Oncol 19:492–501

    Article  PubMed  CAS  Google Scholar 

  • Biegel JA, Rorke LB, Packer RJ, Emanuel BS (1990) Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 73:710–714

    Article  PubMed  CAS  Google Scholar 

  • Biegel JA, Zhou JY, Rorke LB, Stenstrom C, Wainwright LM, Fogelgren B (1999) Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59:74–79

    PubMed  CAS  Google Scholar 

  • Biegel JA, Fogelgren B, Zhou JY, James CD, Janss AJ, Allen JC, Zagzag D, Raffel C, Rorke LB (2000) Mutations of the INI1 rhabdoid tumor suppressor gene in medulloblastomas and primitive neuroectodermal tumors of the central nervous system. Clin Cancer Res 6:2759–2763

    PubMed  CAS  Google Scholar 

  • Biegel JA, Tan L, Zhang F, Wainwright L, Russo P, Rorke LB (2002) Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extra-renal rhabdoid tumors. Clin Cancer Res 8:3461–3467

    PubMed  CAS  Google Scholar 

  • Briner J, Bannwart F, Kleihues P (1985) Malignant small cell tumor of the brain with intermediate filaments – a case of primary cerebral rhabdoid tumor. Pediatr Pathol 3:117–118

    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: a Pediatric Oncology Group study. Am J Surg Pathol 22:1083–1092

    Article  PubMed  CAS  Google Scholar 

  • Cheng YC, Lirng JF, Chang FC, Guo WY, Teng MM, Chang CY, Wong TT, Ho DM (2005) Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol 46:89–96

    Article  PubMed  CAS  Google Scholar 

  • Erickson ML, Johnson R, Bannykh SI, de Lotbiniere A, Kim JH (2005) Malignant rhabdoid tumor in a pregnant adult female: literature review of central nervous system rhabdoid tumors. J Neurooncol 74:311–319

    Article  PubMed  Google Scholar 

  • Fisher BJ, Siddiqui J, Macdonald D, Cairney AE, Ramsey D, Munoz D, Del Maestro R (1996) Malignant rhabdoid tumor of the brain: an aggressive clinical entity. Can J Neurol Sci 23:257–263

    PubMed  CAS  Google Scholar 

  • Gandhi CD, Krieger MD, McComb JG (2004) Atypical teratoid/rhabdoid tumor: an unusual presentation. Neuroradiology 46:834–837

    Article  PubMed  Google Scholar 

  • Gyure KA (2005) Newly defined central nervous system neoplasms. Am J Clin Pathol 123:S3–S12

    PubMed  Google Scholar 

  • Haas JE, Palmer NF, Weinberg AG, Beckwith JB (1981) Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol 12:646–657

    Article  PubMed  CAS  Google Scholar 

  • Hilden JM, Watterson J, Longee DC, Moertel CL, Dunn ME, Kurtzberg J, Scheithauer BW (1998) Central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature. J Neurooncol 40:265–275

    Article  PubMed  CAS  Google Scholar 

  • Hilden JM, Meerbaum S, Burger P, Finlay J, Janss A, Scheithauer BW, Walter AW, Rorke LB, Biegel JA (2004) Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 22:2877–2884

    Article  PubMed  Google Scholar 

  • Hirth A, Pedersen PH, Baardsen R, Larsen JL, Krossnes BK, Helgestad J (2003) Gamma-knife radiosurgery in paediatric cerebral and skull base tumors. Med Pediatr Oncol 40:99–103

    Article  PubMed  Google Scholar 

  • Kababri ME, Andre N, Carole C, Lena G, Figarella-Branger D, Gentet JC (2006) Atypical teratoid rhabdoid tumor in a child with neurofibromatosis-1. Pediatr Blood Cancer 46:267–268

    Article  PubMed  Google Scholar 

  • Kao CL, Chiou SH, Chen YJ, Singh S, Lin HT, Liu RS, Lo CW, Yang CC, Chi CW, Lee CH, Kaya B, Mena H, Miettinen M, Rushing EJ (2003) Alpha-internexin expression in medulloblastoma and atypical teratoid-rhabdoid tumors. Clin Neuropathol 22:215–221

    Google Scholar 

  • Kao CL, Chiou SH, Chen YJ, Singh S, Lin HT, Liu RS, Lo CW, Yang CC, Chi CW, Lee CH, Wong TT (2005) Increased expression of osteopontin gene in atypical teratoid/rhabdoid tumor of the central nervous system. Mod Pathol 18:769–778

    Google Scholar 

  • Oka H, Scheithauer BW (1999) Clinicopathological characteristics of atypical teratoid/rhabdoid tumor. Neurol Med Chir (Tokyo) 39:510–518

    Article  CAS  Google Scholar 

  • Olson TA, Bayar E, Kosnik E, Hamoudi AB, Klopfenstein KJ, Pieters RS, Ruymann FB (1995) Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Pediatr Hematol Oncol 17:71–75

    Article  PubMed  CAS  Google Scholar 

  • Packer RJ, Biegel JA, Blaney S, Finlay J, Geyer JR, Heideman R, Hilden J, Janss AJ, Kun L, Vezina G, Rorke LB, Smith M (2002) Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol 24:337–342

    Article  PubMed  Google Scholar 

  • Parwani AV, Stelow EB, Pambuccian SE, Burger PC, Ali SZ (2005) Atypical teratoid/rhabdoid tumor of the brain: cytopathologic characteristics and differential diagnosis. Cancer 105:65–70

    Article  PubMed  Google Scholar 

  • Rickert CH, Paulus W (2001) Epidemiology of central nervous system tumors in childhood and adolescence based on new WHO classification. Childs Nerv Syst 17:503–511

    Article  PubMed  CAS  Google Scholar 

  • Roberts CW, Galusha SA, McMenabin ME, Fletcher CD, Orkin SH (2000) Haploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice. Proc Natl Acad Sci USA 97:13796–13800

    Article  PubMed  CAS  Google Scholar 

  • Rorke LB, Packer R, Biegel J (1995) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. J Neurooncol 24:21–28

    Article  PubMed  CAS  Google Scholar 

  • 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 

  • Sainte-Rose C, Cinalli G, Roux FE, Maixner R, Chumas PD, Mansour M, Carpentier A, Bourgeois M, Zerah M, Pierre-Kahn A, Renier D (2001) Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy. J Neurosurg 95:791–797

    Article  PubMed  CAS  Google Scholar 

  • Strother D (2005) Atypical teratoid rhabdoid tumors of childhood: diagnosis, treatment and challenges. Expert Rev Anticancer Ther 5:907–915

    Article  PubMed  Google Scholar 

  • Taylor MD, Gokgoz N, Andrulis IL, Mainprize TG, Drake JM, Rutka JT (2000) Familial posterior fossa brain tumors of infancy secondary to germ-line mutation of the hSNF5 gene. Am J Hum Genet 66:1403–1406

    Article  PubMed  CAS  Google Scholar 

  • Tekautz TM, Fuller CE, Blaney S, Fouladi M, Bronicer A, Merchant TE, Krasin M, Dalton J, Hale G, Kun LE, Wallace D, Gilbertson RJ, Gujjar A (2005) Atypical teratoid/rhabdoid tumors (AT/RT): improved survival in children 3 years of age and older with radiation therapy and high dose alkylator based chemotherapy. J Clin Oncol 23:1491–1499

    Article  PubMed  CAS  Google Scholar 

  • Versteege I, Sevenet N, Lange J, Rousseau-Merck MF, Ambros P, Handgretinger R, Aurias A, Delattre O (1998) Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature 394:203–206

    Article  PubMed  CAS  Google Scholar 

  • Wong TT (2005) Increased expression of osteopontin gene in atypical teratoid/rhabdoid tumor of the central nervous system. Mod Pathol 18:769–778

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India

    Krishan Kumar Bansal & Deepak Goel

Authors
  1. Krishan Kumar Bansal
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Deepak Goel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Krishan Kumar Bansal .

Editor information

Editors and Affiliations

  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Bansal, K.K., Goel, D. (2012). Pediatric Atypical Teratoid/Rhabdoid Tumors (An Overview). In: Hayat, M. (eds) Pediatric Cancer, Volume 2. Pediatric Cancer, vol 2. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2957-5_1

Download citation

Publish with us

Policies and ethics

Search

Navigation