Update on childhood craniopharyngiomas.

Area of Science:

• Neuro-oncology
• Neurosurgery
• Endocrinology

Background:

• Craniopharyngioma location significantly impacts treatment strategies and patient prognosis.
• Advances in neuroimaging enhance anatomical localization for surgical planning.
• Recent research deepens the understanding of craniopharyngioma pathogenesis and therapeutic options.

Purpose of the Study:

• To review recent advancements in craniopharyngioma treatment approaches based on anatomical location.
• To highlight the role of imaging in surgical planning and risk assessment.
• To discuss emerging targeted therapies and their potential to mitigate treatment-related morbidities.

Main Methods:

• Literature review of publications from January 2015 to March 2016, including prior key reports.
• Analysis of imaging findings for anatomical localization and risk stratification.
• Review of molecular findings and their implications for targeted therapies.

Main Results:

• Third ventricular and hypothalamic involvement correlate with the highest risk of postoperative hypothalamic dysfunction.
• Presurgical grading using clinical presentation and MRI aids in predicting and potentially limiting morbidities like obesity and neurocognitive deficits.
• Papillary craniopharyngiomas (14-50% of adult cases) often harbor BRAF mutations, suggesting sensitivity to BRAF and MEK inhibitors.
• Adamantinomatous craniopharyngiomas (remaining adult and majority of childhood cases) frequently show CTNNB1 mutations, activating WNT signaling.

Conclusions:

• Craniopharyngioma is associated with significant morbidities, necessitating careful treatment planning.
• Targeted medical therapies are emerging as promising options to complement or refine treatment strategies.
• Surgical and radiation therapy decisions must prioritize minimizing long-term sequelae, especially hypothalamic dysfunction.