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Posterior fossa tumors in children: current insights
Cleiton Formentin1,2, Andrei Fernandes Joaquim3,4, Enrico Ghizoni3,4
1Division of Neurosurgery, Department of Neurology, University of Campinas, Tessalia Vieira de Camargo St., 126. 13083-887, Campinas, SP, Brazil. cleitonformentin@gmail.com.
Insights
Pediatric posterior fossa tumors are increasingly diagnosed and treated using molecular biomarkers. These advancements improve patient outcomes and personalize treatment strategies for childhood brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Molecular pathology of CNS tumors
- Childhood brain tumor classification
Background:
- Posterior fossa tumors constitute a significant proportion of pediatric central nervous system (CNS) neoplasms, despite representing a small fraction of intracranial volume.
- The World Health Organization (WHO) classification of CNS tumors has undergone significant revisions, impacting the understanding and categorization of pediatric posterior fossa tumors.
- Traditional management relies on multimodal approaches, including surgery, radiation, and chemotherapy, often guided by the extent of tumor resection.
Purpose of the Study:
- To review recent advancements in the diagnosis and treatment of common pediatric posterior fossa tumors.
- To highlight the evolving role of molecular biomarkers in understanding, classifying, and managing these tumors.
- To explore future therapeutic strategies informed by molecular profiling and targeted treatments.
Main Methods:
- Review of current literature on pediatric posterior fossa tumors.
- Analysis of recent updates in WHO CNS tumor classification.
- Examination of the application of molecular biomarkers in diagnosis, prognosis, and treatment selection.
Main Results:
- Molecular biomarkers are crucial for accurate diagnosis, prognosis prediction, and tailoring treatment for pediatric posterior fossa tumors.
- Advances in molecular stratification enable personalized therapies targeting specific tumor characteristics.
- Improved neuroimaging and treatment modalities have led to better survival rates and quality of life for affected children.
Conclusions:
- Molecular biomarkers are central to the diagnosis, prognosis, and customized treatment of pediatric posterior fossa tumors.
- These biomarkers aid in predicting outcomes, monitoring treatment response, and assessing therapeutic effectiveness.
- Progress in neuroimaging and therapeutic interventions significantly enhances outcomes for children with these tumors.
Abstract:
While in adults most intracranial tumors develop around the cerebral hemispheres, 45 to 60% of pediatric lesions are found in the posterior fossa, although this anatomical region represents only 10% of the intracranial volume. The latest edition of the WHO classification for CNS tumors presented some fundamental paradigm shifts that particularly affected the classification of pediatric tumors, also influencing those that affect posterior fossa. Molecular biomarkers play an important role in the diagnosis, prognosis, and treatment of childhood posterior fossa tumors and can be used to predict patient outcomes and response to treatment and monitor its effectiveness. Although genetic studies have identified several posterior fossa tumor types, differing in terms of their location, cell of origin, genetic mechanisms, and clinical behavior, recent management strategies still depend on uniform approaches, mainly based on the extent of resection. However, significant progress has been made in guiding therapy decisions with biological or molecular stratification criteria and utilizing molecularly targeted treatments that address specific tumor biological characteristics. The primary focus of this review is on the latest advances in the diagnosis and treatment of common subtypes of posterior fossa tumors in children, as well as potential therapeutic approaches in the future. Conclusion: Molecular biomarkers play a central role, not only in the diagnosis and prognosis of posterior fossa tumors in children but also in customizing treatment plans. They anticipate patient outcomes, measure treatment responses, and assess therapeutic effectiveness. Advances in neuroimaging and treatment have significantly enhanced outcomes for children with these tumors. What is Known: • Central nervous system tumors are the most common solid neoplasms in children and adolescents, with approximately 45 to 60% of them located in the posterior fossa. • Multimodal approaches that include neurosurgery, radiation therapy, and chemotherapy are typically used to manage childhood posterior fossa tumors What is New: • Notable progress has been achieved in the diagnosis, categorization and management of posterior fossa tumors in children, leading to improvement in survival and quality of life.
