Multidisciplinary Management of Pediatric Craniopharyngioma: From Diagnosis to Long-Term Outcomes.
This narrative review summarizes multidisciplinary pediatric craniopharyngioma management, highlighting molecular subtypes, anatomy-guided hypothalamus-sparing surgery, modern radiotherapy, and emerging targeted and intracystic treatments aimed at preserving long-term function.
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This narrative review summarizes multidisciplinary pediatric craniopharyngioma management, highlighting molecular subtypes, anatomy-guided hypothalamus-sparing surgery, modern radiotherapy, and emerging targeted and intracystic treatments aimed at preserving long-term function.
Research significance
The review reports that risk-adapted surgery— including subtotal resection with modern radiotherapy in selected patients—may preserve hypothalamic and other functions while maintaining tumor control, and it identifies subtype-associated molecular alterations as potential therapeutic entry points; however, the supplied record does not provide primary comparative or trial evidence establishing the safety or efficacy of these strategies.
Source abstract
Background: Pediatric craniopharyngiomas are rare, histologically differentiated epithelial tumors arising in the sellar-suprasellar region. Despite their low-grade classification, these tumors are associated with significant long-term morbidity because of their proximity to critical neurovascular and hypothalamic structures. Management remains challenging and requires a careful balance between durable tumor control and the preservation of neurological, endocrine, and neurocognitive functions. Methods: This narrative review summarizes the current evidence concerning the epidemiology, molecular pathogenesis, clinical presentation, diagnostic evaluation, and treatment strategies for pediatric craniopharyngiomas. Emphasis is placed on anatomical and hypothalamic involvement-based classifications, evolving surgical philosophies, and the role of multimodal management. Results: Advances in molecular characterization have resulted in the delineation of two distinct subtypes-adamantinomatous and papillary craniopharyngiomas-with different biological drivers and therapeutic implications. Surgical management has evolved from historically aggressive gross total resection to risk-adapted, anatomy-driven approaches that prioritize hypothalamic preservation. In selected patients, subtotal resection combined with modern radiotherapy provides tumor control comparable to that of radical surgery, with reduced morbidity. Endoscopic endonasal and microsurgical transcranial approaches play complementary roles, with approach selection guided by tumor anatomy, hypothalamic involvement, and surgical expertise. Emerging targeted therapies and intracystic treatments further expand therapeutic options. Conclusions: The contemporary management of pediatric craniopharyngiomas emphasizes individualized, multidisciplinary strategies focused on functional preservation rather than anatomical radicality alone. Integration of molecular insights, advanced imaging, and long-term outcome data is essential to further improve survival and quality of life in this vulnerable population.