Intracranial germ cell tumors in Adolescents and Young Adults: European and North American consensus review, current management and future development.
Faure Conter, C
Oxford University Press (OUP)
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Frappaz, D., Dhall, G., Murray, M., Goldman, S., Faure Conter, C., Allen, J., Kortmann, R., et al. (2021). Intracranial germ cell tumors in Adolescents and Young Adults: European and North American consensus review, current management and future development.. Neuro Oncol https://doi.org/10.1093/neuonc/noab252
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.
Brain tumors, Germ Cell Tumor, Germinoma, Non-germinomatous germ cell tumor, adolescent and young adult
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External DOI: https://doi.org/10.1093/neuonc/noab252
This record's URL: https://www.repository.cam.ac.uk/handle/1810/330476
Attribution-NonCommercial 4.0 International
Licence URL: https://creativecommons.org/licenses/by-nc/4.0/