Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (01/24/2018)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Tumor Biology of Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor (AT/RT)

Added Cribriform Neuroepithelial Tumor as a new subsection.

Treatment of Newly Diagnosed Childhood CNS AT/RT

Added text to state that a report using the National Cancer Database described the outcome of 361 children with CNS AT/RT who were aged 0 to 18 years at diagnosis and treated in two periods. The report confirmed the increased use of trimodal therapy during the study period and an improvement in outcome (cited Fischer-Valuck et al. as reference 13).

Treatment of Recurrent Childhood CNS AT/RT

Added text to state that in a report of four children with recurrent AT/RT treated with an aurora kinase inhibitor, three children responded; the responses were sustained for more than 1 year (cited Wetmore et al. as reference 1). Also added text to state that a larger trial (NCT02114229) is underway.

Added text about the NCT02114229 trial as a treatment option under clinical evaluation for recurrent childhood CNS AT/RT.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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