Atypical teratoid/rhabdoid tumor of the central nervous system

Report on workshop

Roger J. Packer, Jaclyn A. Biegel, Susan Blaney, Jonathan Finlay, J. Russell Geyer, Richard Heideman, Joanne Hilden, Anna J. Janss, Larry Kun, Gilbert Vezina, Lucy B. Rorke, Malcolm Smith

Research output: Contribution to journalArticle

173 Citations (Scopus)

Abstract

Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity. Diagnosis is based on distinctive light microscopy and immunohistochemical findings, coupled with molecular genetic analysis. Most AT/RTs demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene. The tumor's incidence is still undefined, but it may comprise as high as 1 in 4 primitive CNS tumors in infants. Treatment is far from optimal, but there are occasional long-term survivors, especially among older children. Therapeutic approached have included surgery, chemotherapy, and radiotherapy. Prospective clinical trials are needed for children with AT/RTs.

Original languageEnglish (US)
Pages (from-to)337-342
Number of pages6
JournalJournal of Pediatric Hematology/Oncology
Volume24
Issue number5
DOIs
StatePublished - Jul 25 2002

Fingerprint

Nervous System Neoplasms
Central Nervous System
Education
Monosomy
Central Nervous System Neoplasms
Chromosome Deletion
Survivors
Molecular Biology
Microscopy
Radiotherapy
Clinical Trials
Light
Drug Therapy
Incidence
Therapeutics
Genes
Neoplasms
Atypical Teratoid Tumor
Typical Teratoid Rhabdoid Tumor

Keywords

  • Atypical teratoid tumors
  • Chemotherapy
  • Infantile brain tumors
  • Radiotherapy
  • Rhabdoid tumors

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Packer, R. J., Biegel, J. A., Blaney, S., Finlay, J., Geyer, J. R., Heideman, R., ... Smith, M. (2002). Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop. Journal of Pediatric Hematology/Oncology, 24(5), 337-342. https://doi.org/10.1097/00043426-200206000-00004

Atypical teratoid/rhabdoid tumor of the central nervous system : Report on workshop. / Packer, Roger J.; Biegel, Jaclyn A.; Blaney, Susan; Finlay, Jonathan; Geyer, J. Russell; Heideman, Richard; Hilden, Joanne; Janss, Anna J.; Kun, Larry; Vezina, Gilbert; Rorke, Lucy B.; Smith, Malcolm.

In: Journal of Pediatric Hematology/Oncology, Vol. 24, No. 5, 25.07.2002, p. 337-342.

Research output: Contribution to journalArticle

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', Journal of Pediatric Hematology/Oncology, vol. 24, no. 5, pp. 337-342. https://doi.org/10.1097/00043426-200206000-00004
Packer, Roger J. ; Biegel, Jaclyn A. ; Blaney, Susan ; Finlay, Jonathan ; Geyer, J. Russell ; Heideman, Richard ; Hilden, Joanne ; Janss, Anna J. ; Kun, Larry ; Vezina, Gilbert ; Rorke, Lucy B. ; Smith, Malcolm. / Atypical teratoid/rhabdoid tumor of the central nervous system : Report on workshop. In: Journal of Pediatric Hematology/Oncology. 2002 ; Vol. 24, No. 5. pp. 337-342.
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