Nemes K, Bens S, Kachanov D, et al. - In 2009, the EU-RHAB registry was created to prospectively obtain data of patients with rhabdoid tumor managed according to the EU-RHAB therapeutic framework. There were 100 patients recruited within the EU-RHAB (2009–2018) for evaluation; these comprised 70 patients presenting with extracranial, extrarenal tumors (eMRT) and 30 with renal rhabdoid tumors (RTK). Significant negative prognostic factors were presence of germline mutations (GLM), metastases at diagnosis (M+) and lack of gross total resection (GTR). Patients with M0 and GTR+ and without GLM defined the standard risk (SR) group. Patients with M+ and/or lack of GTR and/or GLM constituted the high risk (HR) group. Five-year OS rates were significantly superior for the SR group (72.2%). Standard conventional chemotherapy was identified as beneficial for SR patients; there is a necessity for novel therapeutic strategies for HR patients.