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

Changes to This Summary (01/28/2019)

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 text about the loss of SMARCB1 or SMARCA4 protein expression and the results of a study that treated adult patients with an EZH2 inhibitor (cited Wilson et al., Knutson et al., Kurmasheva et al., and Italiano et al. as references 20, 21, 22, and 23, respectively).

Treatment of Recurrent Childhood CNS AT/RT

Added text to state that in a study of the EZH2 inhibitor tazemetostat in adult patients with epithelioid sarcoma and non-CNS malignant rhabdoid tumors with SMARCB1 or SMARCA4 loss, prolonged stable disease and objective responses were observed (cited Italiano et al. as reference 1). Also added text to state that the activity of tazemetostat in children with AT/RT is under clinical evaluation.

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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