What changed: Atypical teratoid/rhabdoid tumour (ATRT)

When this page itself was last checked or edited.

Actionable alteration in 31.

Maximal safe resection followed by an intensive multimodal protocol: ACNS0333-style induction, high-dose chemotherapy with autologous stem-cell rescue, and age-adapted focal radiotherapy; or the EU-RHAB regimen with intraventricular methotrexate. Enrolment in SIOPE ATRT01 or a COG successor where available.

Hazard of an event 0.

COG phase 3 with high-dose chemotherapy and autologous rescue (JCO 2020).

First EZH2 inhibitor approval; paediatric rhabdoid tumour responses in the phase 1 programme.

Johann and Torchia (Cancer Cell) describe ATRT-TYR, ATRT-SHH and ATRT-MYC by methylation and expression.

Versteege and colleagues (Nature) find biallelic loss, the first chromatin-remodelling tumour suppressor.

Rorke and colleagues recognise the rhabdoid tumour of the CNS.