The incidence of intracranial germ cell tumors (iCGT) is much lower in European and North American (E&NA) than in Asian population. However, E&NA cooperative groups have successfully developed in parallel treatment strategies with specific attention paid to long-term sequelae. Neurological sequelae may be reduced by establishing a diagnosis with an endoscopic biopsy and/or CSF and/or serum analysis, deferring the need to perform a radical surgery. Depending on markers and/or histological characteristics, patients are treated either as germinoma, or as non-germinomatous germ cell tumors (NGGCT). Metastatic disease is defined by a positive CSF cytology and/or distant drops in cranio-spinal MRI. The combination of surgery and/or chemotherapy and radiation therapy is tailored according to grouping and staging. With more than 90% 5-year event-free survival (EFS), localized germinomas can be managed without aggressive surgery, and benefit from chemotherapy followed by whole ventricular irradiation with local boost. Bifocal germinomas are treated as non-metastatic entities. Metastatic germinomas may be cured with craniospinal irradiation.. With a 5-year EFS over 70%, NGGCT benefit from chemotherapy followed by delayed surgery in case of residual disease, and some form of radiotherapy. Future strategies will aim at decreasing long-term side effects while preserving high cure rates.
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D Frappaz, G Dhall, M J Murray, S Goldman, C Faure Conter, J Allen, R Kortmann, D Haas-Kogen, G Morana, J Finlay, J C Nicholson, Ute Bartels, M Souweidane, S Schöenberger, A Vasiljevic, P Robertson, A Albanese, C Alapetite, T Czech, C C Lau, P Wen, D Schiff, D Shaw, G Calaminus, E Bouffet, Intracranial germ cell tumors in Adolescents and Young Adults: European and North American consensus review, current management and future development, Neuro-Oncology, 2021;, noab252, https://doi.org/10.1093/neuonc/noab252