Advances in Pediatric Neuro-Oncology in the Molecular Era (218)

4:15–5:15 pm Friday, September 6

Pediatric CNS tumors as a group represent the most common solid tumor in childhood and encompass a wide variety of diagnoses, from benign to malignant. Recent advances in molecular profiling have led to significant changes in the classification of pediatric CNS tumors, with a positive impact on diagnosis, prognosis, and treatment. Gliomas have undergone reclassification with emphasis on molecular profile over histology, leading to a redefinition of traditional low-grade versus high-grade glioma.

Understanding of tumor mutations in low-grade gliomas has led to use of new targeted therapies that can improve outcome. For high-grade gliomas, there is increasing interest in the use of immunotherapies. Medulloblastoma and ependymoma have newly identified subgroups. Within medulloblastoma, the new subgroups are leading to potential changes in treatment with the hope for less toxicity and improved quality of life for survivors. Embryonal tumors as a group have also seen considerable reclassification based on molecular findings, with the removal of the term PNET from the lexicon in the 2016 WHO classification of CNS tumors, and the emergence of new groups of embryonal tumors, such as embryonal tumor with multilayered rosettes (ETMR). Altogether these changes have increased our understanding on the course of the disease, variability in outcome, and appropriate treatments for different groups and subgroups of pediatric CNS tumors. General oncology practitioners and neuro-oncology specialists alike must learn to integrate our traditional understanding of pediatric CNS tumors with new molecular findings in order to provide the highest quality of information and care to our patients and families.

Speaker(s):
Cheryl Fischer, MSN RN CPNP
CNE Hours:
1