The incidence of intracranial germ cell tumors (iGCT) is much lower in European and North American (E&NA) than in Asian population. However, E&NA cooperative groups have simultaneously developed with success 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 cerebrospinal fluid (CSF) and/or serum analysis, deferring the need to perform a radical surgery. Depending on markers and/or histological characteristics, patients are treated as either germinoma or non-germinomatous germ cell tumors (NGGCT). Metastatic disease is defined by a positive CSF cytology and/or distant drops in craniospinal 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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Didier Frappaz, Girish Dhall, Matthew J Murray, Stuart Goldman, Cecile Faure Conter, Jeffrey Allen, Rolf Dieter Kortmann, Daphne Haas-Kogen, Giovanni Morana, Jonathan Finlay, James C Nicholson, Ute Bartels, Mark Souweidane, Stefan Schönberger, Alexandre Vasiljevic, Patricia Robertson, Assunta Albanese, Claire Alapetite, Thomas Czech, Chin C Lau, Patrick Wen, David Schiff, Dennis Shaw, Gabriele Calaminus, Eric Bouffet, EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults, Neuro-Oncology, Volume 24, Issue 4, April 2022, Pages 516–527, doi.org/10.1093/neuonc/noab252