Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK)

•Presence of GLM, M+ and lack of GTR are significant negative prognostic factors.•A Standard risk’ (SR) group contains patients with M0 and GTR+ and without GLM.•A ‘High risk’ (HR) group presents patients with M+ and/or lack of GTR and/or GLM.•The SR group demonstrated significantly superior 5-year OS rates (72.2%).•SR patients benefit from standard conventional chemotherapy, HR patients need novel therapeutic strategies.