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Central nervous system germ cell tumors: an update.
Christelle Dufour1, Léa Guerrini-Rousseau, Jacques Grill
1Brain Tumor Programme, Department of Pediatric and Adolescent Oncology, Gustave Roussy and University Paris-Sud, Villejuif, France.
Current Opinion in Oncology
|September 19, 2014
Summary
Malignant germ cell tumors of the brain are rare but treatable with established multidisciplinary care. Advances in chemotherapy and potential targeted therapies offer improved outcomes, especially for secreting neoplasms.
Area of Science:
- Neuro-oncology
- Pediatric Oncology
- Genomics
Background:
- Malignant germ cell tumors (GCTs) of the central nervous system (CNS) are rare, primarily affecting adolescents.
- Their rarity leads to dispersal across pediatric and adult wards, complicating management and research.
- Recent high-throughput genomics are beginning to elucidate their biology.
Purpose of the Study:
- To review the current understanding of CNS malignant germ cell tumors.
- To highlight advancements in diagnosis and treatment strategies.
- To discuss emerging therapeutic targets based on molecular findings.
Main Methods:
- Review of international studies and clinical trials.
- Analysis of genomic data from GCT patients.
- Evaluation of treatment outcomes including chemotherapy and radiotherapy.
Main Results:
- Chemotherapy is crucial for tumor control in secreting GCTs and can reduce radiation doses in germinomas.
- Neurocognitive outcomes are generally good, with variations based on tumor location (pineal vs. supratentorial).
- Frequent alterations in KIT/RAS and mTOR/AKT pathways suggest potential for targeted therapies.
Conclusions:
- Diagnosis and treatment are well-established for adolescents and young adults within multidisciplinary teams.
- Aggressive chemotherapy, including high-dose regimens with stem-cell support, may improve outcomes for secreting GCTs.
- The role of targeted therapies requires further investigation based on recent molecular discoveries.

