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Pediatric Central Nervous System Cancers, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology
Amar Gajjar1, Anita Mahajan2, Mohamed Abdelbaki3
11St. Jude Children's Research Hospital/The University of Tennessee Health Science Center.
Abstract:
Central nervous system (CNS) cancers account for approximately one quarter of all pediatric tumors and are the leading cause of cancer-related death in children. More than 4,000 brain and CNS tumors are diagnosed each year in children and teens, and the incidence rate has remained stagnant in recent years. The most common malignant pediatric CNS tumors are gliomas, embryonal tumors consisting of predominately medulloblastomas, and germ cell tumors. The inaugural version of the NCCN Guidelines for Pediatric Central Nervous System Cancers focuses on the diagnosis and management of patients with pediatric diffuse high-grade gliomas. The information contained in the NCCN Guidelines is designed to help clinicians navigate the complex management of pediatric patients with diffuse high-grade gliomas. The prognosis for these highly aggressive tumors is generally poor, with 5-year survival rates of <20% despite the use of combined modality therapies of surgery, radiation therapy and systemic therapy. Recent advances in molecular profiling has expanded the use of targeted therapies in patients whose tumors harbor certain alterations. However, enrollment in a clinical trial is the preferred treatment for eligible patients.
Insights
Pediatric central nervous system (CNS) cancers are a leading cause of childhood cancer death. New guidelines aid clinicians in managing aggressive diffuse high-grade gliomas, offering hope through targeted therapies and clinical trials.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Clinical Guidelines
Background:
- Central nervous system (CNS) cancers represent a significant portion of pediatric tumors, causing substantial mortality.
- Diffuse high-grade gliomas are particularly aggressive pediatric CNS tumors with poor prognoses.
Purpose of the Study:
- To introduce the NCCN Guidelines for Pediatric Central Nervous System Cancers, focusing on diffuse high-grade gliomas.
- To provide clinicians with a framework for diagnosing and managing pediatric diffuse high-grade gliomas.
Main Methods:
- Development of clinical guidelines based on current evidence and expert consensus.
- Focus on diagnosis and management strategies for pediatric diffuse high-grade gliomas.
Main Results:
- The guidelines offer a structured approach to the complex care of pediatric diffuse high-grade gliomas.
- Current treatments yield poor survival rates (<20% at 5 years) despite multimodal therapy.
Conclusions:
- Molecular profiling is enhancing the use of targeted therapies for specific tumor alterations.
- Clinical trial enrollment is recommended as the preferred treatment option for eligible pediatric patients.
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