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

doi:10.1097/00043426-200206000-00004

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 journal › Article › peer-review

214 Scopus citations

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 language	English (US)
Pages (from-to)	337-342
Number of pages	6
Journal	Journal of Pediatric Hematology/Oncology
Volume	24
Issue number	5
DOIs	https://doi.org/10.1097/00043426-200206000-00004
State	Published - 2002

Keywords

Atypical teratoid tumors
Chemotherapy
Infantile brain tumors
Radiotherapy
Rhabdoid tumors

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1097/00043426-200206000-00004

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title = "Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop",

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.",

keywords = "Atypical teratoid tumors, Chemotherapy, Infantile brain tumors, Radiotherapy, Rhabdoid tumors",

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

year = "2002",

doi = "10.1097/00043426-200206000-00004",

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pages = "337--342",

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AU - Packer, Roger J.

AU - Biegel, Jaclyn A.

AU - Blaney, Susan

AU - Finlay, Jonathan

AU - Geyer, J. Russell

AU - Heideman, Richard

AU - Hilden, Joanne

AU - Janss, Anna J.

AU - Kun, Larry

AU - Vezina, Gilbert

AU - Rorke, Lucy B.

AU - Smith, Malcolm

PY - 2002

Y1 - 2002

N2 - 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.

AB - 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.

KW - Atypical teratoid tumors

KW - Chemotherapy

KW - Infantile brain tumors

KW - Radiotherapy

KW - Rhabdoid tumors

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ER -

Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop

Abstract

Keywords

ASJC Scopus subject areas

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