CNS-InterREST

Primary study objectives

• Clinical advice to local practitioners within the GPOH and internationally according to the latest scientific findings
• Prompt interdisciplinary discussion of patients in the CNS-InterREST Tumor Board (currently every 14 days on Thursdays)
• Prospective documentation of tumor type-specific clinical, therapeutic and outcome data
• Further development of the classification of rare CNS tumors through neuropathological reference diagnostics according to the current WHO standard and their molecular-diagnostic processing
• Development of tumor type-specific diagnostic and therapeutic strategies
• Simplification of the establishment of early clinical studies for these rare entities

Prospective and retrospective international registry

All patients with rare embryonal or sarcomatous CNS tumors from all participating centers in the participating countries are to be recorded in the CNS-InterREST registry. The aim is to comprehensively document the histomorphological, molecular genetic and epigenetic characteristics of the above-mentioned rare tumor types in children and adolescents. Furthermore, clinical data, image data, the course of therapy and the response to the selected therapy regime, as well as outcome data (OS/PFS) are to be recorded.

Treatment recomendations

The CNS-InterREST registry serves exclusively to collect data; no explicit treatment recommendations are made. As there are currently no standard therapies for these rare entities, the study management is available on request for clinical consultation and discussion of patients in the interdisciplinary CNS-InterREST Tumor Board.

At this point, we would also like to refer you to the ESCP Guidelines: Rare CNS embryonal and sarcomatous tumors and Astroblastoma, MN1-altered on the SIOPE portal https://siope.eu/media/documents/escp-rare-cns-embryonal-and-sarcomatous-tumours.pdf

• Patients with an integrated morphological and molecular diagnosis or a rare embryonal or sarcomatous brain tumor (ETMR; CNS-NB FOXR2; CNS BCOR-ITD; CNS-CIC; CNS-DICER1; Astroblastoma-MN1, etc.); a higher-grade embryonal or sarcomatous tumor not elsewhere classified (NEC); a molecularly newly defined entity from this spectrum
• Possibility of reference neuropathological diagnosis, i.e. sufficient available tumor material (FFPE min. 3 x 3 mm) or available DNA methylation data (IDAT files)
• Age under 18 years (note: this registry and the advice provided by the study group is clearly focused on the pediatric population, but inclusion of adult patients with one of the above-mentioned rare diagnoses is possible in individual cases)
• Localization of the primary tumor in the CNS
• Patients with all clinical stages can be included.
• Existing informed consent from the patient or legal guardian
• Inclusion is possible at the time of initial diagnosis, relapse diagnosis or at any time during follow-up care.

• Lack of informed consent
• Lack of sufficient tumor material or DNA methylation (raw) data (IDAT files) for reference diagnostics
• Integrated neuropathological diagnosis of a medulloblastoma
• Integrated neuropathological diagnosis of an AT/RT or cribriform neuroepithelial tumor
• Integrated neuropathological diagnosis of an ependymal tumor
• Integrated neuropathologic diagnosis of a glioma