[Craniopharyngioma : Current Perspectives Including Recent Advances in Molecular Targeted Therapy]

Craniopharyngiomas are benign sellar and parasellar tumors and clinically challenging to treat because of their proximity to the critical neurovascular and hypothalamic structures. Recently, distinct driver mutations, including CTNNB1 in adamantinomatous craniopharyngiomas and BRAF V600E in papillary craniopharyngiomas, have led to a paradigm shift in treatment strategies. Endoscopic endonasal transsphenoidal surgery, supported by advances in surgical instruments and techniques, has expanded the indications for this minimally invasive surgical strategy, which has become an important approach in contemporary surgical management. Radiotherapy remains an effective adjuvant option for residual or recurrent tumors and contributes to durable local tumor control if combined with function-preserving surgical strategies. Molecular targeted therapy combining BRAF and MEK inhibitors results in marked tumor shrinkage in papillary craniopharyngiomas harboring the BRAF V600E mutation. These agents have emerged as promising therapeutic options, potentially as neoadjuvant therapies for enhancing surgical safety or achieving non-surgical tumor control in selected cases. Future management of craniopharyngiomas requires an individualized, multimodal approach that integrates the molecular subtype, patient age, functional status, and long-term quality of life to choose a treatment course that balances durable tumor control with optimal functional outcomes.