EANO Guideline for intracranial germ cell tumors

EANO, SNO and Euracan guideline for intracranial germ cell tumor (2021)

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