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Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs).

Authors
  • Garrè, Maria Luisa
  • Tekautz, Tanya
Type
Published Article
Journal
Pediatric Blood & Cancer
Publisher
Wiley (John Wiley & Sons)
Publication Date
Apr 01, 2010
Volume
54
Issue
4
Pages
647–648
Identifiers
DOI: 10.1002/pbc.22377
PMID: 20146222
Source
Medline
License
Unknown

Abstract

Atypical teratoid/rhabdoid tumors (AT/RTs) of the CNS have been recently characterized as a distinct clinicopathologic entity with an unusually poor prognosis and with the highest incidence in the first 2 years of life. It often arises in the posterior fossa and its distinctive immunohistochemical (negative stain for INI-1) and cytogenetic features (monosomy or deletion of chromosome 22) permit an adequate diagnosis in most of cases. AT/RT of the CNS is a usually fatal disease virtually unresponsive to chemotherapy (CT) and radiotherapy (RT). Rapid progression and CNS dissemination are commonly reported. Whether combined regimens including high-dose CT are able to prolong survival or change the natural history of this tumor are under evaluation.

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