Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma

ABSTRACT

Craniopharyngiomas are rare embryonic malformations of the sellar/parasellar region with low histological grade. Here, we review findings on the diagnosis, treatment, clinical course, follow-up, and prognosis of craniopharyngioma patients. Clinical manifestations develop from increased intracranial pressure, anterior visual pathway damage, and hypothalamic/pituitary deficiencies. If the tumor is favorably localized (no anatomical involvement with the hypothalamic and optical structures) therapy of choice is complete resection, meticulously performed to preserve hypothalamic and optic functions. In patients with unfavorable tumor involvement, optimal therapy is limited hypothalamus-sparing surgical strategy, followed by judicious irradiation dosage to minimize recurrences and progression. Surgical lesions and/or anatomical involvement of posterior hypothalamic areas result in serious sequelae, mainly hypothalamic syndrome. Craniopharyngioma is a chronic disease and must be managed as such, providing ongoing care of pediatric and adult patients by experienced multidisciplinary teams in the context of multicenter trials.

KEYWORDS:

• Craniopharyngioma
• neurosurgery
• hypothalamic obesity
• daytime sleepiness
• pituitary deficiency
• neurocognitive
• recurrence
• quality of life
• brain tumors
• irradiation

Acknowledgement

The author is grateful for the help of Mrs. Margarita Neff-Heinrich (Göttingen, Germany) in proofreading and editing the manuscript.

Declaration of interest

The author is supported by the German Childhood Cancer Foundation, Bonn, Germany. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.