Role of High-Dose Chemotherapy (HDCT) in treatment of Atypical Teratoid/Rhabdoid Tumors (AT/RTs)

Maria Luisa Garrè, Tanya Tekautz

Research output: Contribution to journalArticle

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 highdose CT are able to prolong survival or change the natural history of this tumor are under evaluation.

Original languageEnglish
Pages (from-to)647-648
Number of pages2
JournalPediatric Blood and Cancer
Volume54
Issue number4
DOIs
Publication statusPublished - Apr 2010

Keywords

  • AT/RT
  • CNS
  • High-dose chemotherapy

ASJC Scopus subject areas

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

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