Supratentorial ependymoma: a comprehensive review of molecular classification, management strategies, and clinical outcomes (Part II of pediatric ependymomas across compartments).
Frederic A Vallejo, Meredith A Monsour, Nathan S Fredricks, Derek S Tsang, George Koutsouras, Timothy A Ritzmann, Kristian Aquilina, Vijay Ramaswamy, Michael C Dewan
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引用次数: 0
Abstract
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.
期刊介绍：
The Journal of Neuro-Oncology is a multi-disciplinary journal encompassing basic, applied, and clinical investigations in all research areas as they relate to cancer and the central nervous system. It provides a single forum for communication among neurologists, neurosurgeons, radiotherapists, medical oncologists, neuropathologists, neurodiagnosticians, and laboratory-based oncologists conducting relevant research. The Journal of Neuro-Oncology does not seek to isolate the field, but rather to focus the efforts of many disciplines in one publication through a format which pulls together these diverse interests. More than any other field of oncology, cancer of the central nervous system requires multi-disciplinary approaches. To alleviate having to scan dozens of journals of cell biology, pathology, laboratory and clinical endeavours, JNO is a periodical in which current, high-quality, relevant research in all aspects of neuro-oncology may be found.
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