Craniopharyngioma – Pediatric Management

Müller, Hermann L.

doi:10.1016/b978-0-12-416706-3.00027-1

Cited by 5 publications

(1 citation statement)

References 183 publications

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“…Energy homeostasis is regulated by a complex neuroendocrine system, of which the hypothalamus is the centre for said regulation. The functional disruption of the hypothalamic network causes hypothalamic obesity [ 1 ▪ , 2 , 3 , 4 ▪ ]. Recent studies have illuminated how the hypothalamus regulates appetite and satiety [ 1 ▪ , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Craniopharyngioma and hypothalamic injury

Müller

2016

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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Purpose of reviewHypothalamic alterations, pathological or treatment induced, have major impact on prognosis in craniopharyngioma patients mainly because of consequent hypothalamic obesity. Recent insight in molecular genetics, treatment strategies, risk factors and outcomes associated with hypothalamic obesity provide novel therapeutic perspectives. This review includes relevant publications since 2013.Recent findingsRecent findings confirm that alterations in posterior hypothalamic areas because of tumour location and/or treatment-related injuries are associated with severe hypothalamic obesity, reduced overall survival and impaired quality of life in long-term survivors of childhood-onset craniopharyngioma. However, eating disorders are observed because of hypothalamic obesity without clear disease-specific patterns. Treatment options for hypothalamic obesity are very limited. Treatment with invasive, nonreversible bariatric methods such as Roux-en-Y gastric bypass is most efficient in weight reduction, but controversial in the paediatric population because of medical, ethical, and legal considerations. Accordingly, treatment in craniopharyngioma should focus on prevention of (further) hypothalamic injury. Presurgical imaging for grading of hypothalamic involvement should be the basis for hypothalamus-sparing strategies conducted by experienced multidisciplinary teams.SummaryUntil a nonsurgical therapeutic option for hypothalamic obesity for paediatric patients is found, prevention of hypothalamic injury should be the preferred treatment strategy, conducted exclusively by experienced multidisciplinary teams.

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