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EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults.

Accepted version
Peer-reviewed

Type

Article

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Authors

Frappaz, Didier 
Dhall, Girish 
Goldman, Stuart 
Faure Conter, Cecile 

Abstract

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.

Description

Keywords

adolescents and young adults, brain tumors, germ cell tumor, germinoma, non-germinomatous germ cell tumor, Adolescent, Brain Neoplasms, Consensus, Germinoma, Humans, Male, Neoplasms, Germ Cell and Embryonal, Retrospective Studies, Testicular Neoplasms, Young Adult

Journal Title

Neuro Oncol

Conference Name

Journal ISSN

1522-8517
1523-5866

Volume Title

Publisher

Oxford University Press (OUP)