Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma. (3rd May 2016)

• Record Type: Journal Article
• Authors: Müller, Hermann L.
• Is Part Of: Expert review of neurotherapeutics. Volume 16:Number 5(2016)
• Page Start: 535
• Page End: 548
• Publication Date: 2016-05-03
• Subjects: Craniopharyngioma -- neurosurgery -- hypothalamic obesity -- daytime sleepiness -- pituitary deficiency -- neurocognitive -- recurrence -- quality of life -- brain tumors -- irradiation; Neuropharmacology -- Periodicals; 615.7805
• DOI: 10.1586/14737175.2016.1166959
• Languages: English
• ISSNs: 1473-7175

Abstract

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.