Childhood craniopharyngioma: current controversies on management in diagnostics, treatment and follow-up

Abstract

Craniopharyngiomas are embryogenic malformations of the sellar region of low histological malignancy, thought to be derived from Rathke’s pouch epithelium. With an overall incidence of 0.5–2.0 new cases per million of the population per year, 30–50% of all cases occur in childhood. Overall survival rates are high. However, quality of survival is substantially reduced in many survivors, owing to long-term sequelae, such as extreme obesity caused by hypothalamic tumor involvement, which occurs in up to 40% of all cases. The treatment of craniopharyngioma remains controversial. Radical surgical strategies are associated with poor outcome in craniopharyngioma with hypothalamic involvement.

Keywords::

• children
• craniopharyngioma
• growth
• hypothalamus
• irradiation
• neurosurgery
• obesity
• pituitary
• quality of life

Acknowledgement

The author expresses particular thanks to Ursel Gebhardt, study assistant of KRANIOPHARYNGEOM 2007, for her help in the writing of the manuscript.

Financial & competing interests disclosure

Supported by the German Pediatric Cancer Foundation (Deutsche Kinderkrebsstiftung), Bonn (www.kinderkrebsstiftung.de). The author declares that no conflict of interest exists according to the Guidelines of the International Committee of Medical Journal Editors. 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.

Writing assistance from Ursel Gebhardt was utilized in the production of this manuscript, but no funding was used.