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

Neuro Oncol. 2022 Apr 1;24(4):516-527. doi: 10.1093/neuonc/noab252.


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.

Keywords: adolescents and young adults; brain tumors; germ cell tumor; germinoma; non-germinomatous germ cell tumor.

MeSH terms

  • Adolescent
  • Brain Neoplasms* / drug therapy
  • Brain Neoplasms* / therapy
  • Consensus
  • Germinoma* / diagnosis
  • Germinoma* / pathology
  • Germinoma* / therapy
  • Humans
  • Male
  • Neoplasms, Germ Cell and Embryonal* / diagnosis
  • Neoplasms, Germ Cell and Embryonal* / therapy
  • Retrospective Studies
  • Testicular Neoplasms*
  • Young Adult