Supratentorial ependymoma: a comprehensive review of molecular classification, management strategies, and clinical outcomes (Part II of pediatric ependymomas across compartments).
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PURPOSE: To review the clinical, molecular, and imaging landscape of pediatric supratentorial ependymoma (ST-EP) and outline current management principles guiding surgical and adjuvant treatment decisions. METHODS: Synthesis of literature regarding biology, diagnosis, and management of ST-EP, focusing on recently emerging molecular subtypes. RESULTS: ST-EP is an important and clinically significant intracranial malignancy comprising roughly 30-40% of all ependymoma cases. The 2021 WHO molecular classification stratified ST-EP into ZFTA-fusion, YAP1-fusion, and NEC subtypes, fundamentally altering our understanding of this pathology. ZFTA-fused tumors represent the majority of ST-EP, are seen in older patients and harbor a relatively worse prognosis while YAP1-fused tumors, though rarer, are enriched for younger children and carry a substantially more favorable prognosis. Management of very young children presents a unique therapeutic challenge, balancing disease control against the neurocognitive sequelae of cranial irradiation and aggressive surgery in the developing brain. CONCLUSION: Across molecular subtypes, maximal safe surgical resection remains the dominant prognostic factor, with adjuvant focal radiotherapy well-supported based on tumor grade and extent of resection. Maintenance chemotherapy following radiotherapy has not demonstrated a survival benefit. Further multi-institutional investigation is needed to formalize molecularly-stratified treatment protocols, better characterize NEC subgroups, and identify targeted therapeutics for high-risk disease. CLINICAL TRIAL NUMBER: Not applicable.