EANO, SNO and Euracan consensus review on the current management and future development of intracranial germ cell tumors in adolescents and young adults. Issue 4 (1st November 2021)

• Record Type: Journal Article
• Authors: Frappaz, Didier; Dhall, Girish; Murray, Matthew J; Goldman, Stuart; Faure Conter, Cecile; Allen, Jeffrey; Kortmann, Rolf Dieter; Haas-Kogen, Daphne; Morana, Giovanni; Finlay, Jonathan; Nicholson, James C; Bartels, Ute; Souweidane, Mark; Schönberger, Stefan; Vasiljevic, Alexandre; Robertson, Patricia; Albanese, Assunta; Alapetite, Claire; Czech, Thomas; Lau, Chin C; Wen, Patrick; Schiff, David; Shaw, Dennis; Calaminus, Gabriele; Bouffet, Eric
• Is Part Of: Neuro-oncology. Volume 24:Issue 4(2022)
• Page Start: 516
• Page End: 527
• Publication Date: 2021-11-01
• Subjects: adolescents and young adults -- brain tumors -- germ cell tumor -- germinoma -- non-germinomatous germ cell tumor; Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noab252
• Languages: English
• ISSNs: 1522-8517

Abstract

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.