Multidisciplinary Management of Pediatric Craniopharyngioma: From Diagnosis to Long-Term Outcomes.

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.