Childhood craniopharyngioma: treatment strategies and outcomes

Expert Rev Neurother. 2014 Feb;14(2):187-97. doi: 10.1586/14737175.2014.875470. Epub 2014 Jan 13.


Craniopharyngiomas are embryogenic malformations of the sellar region. With an overall incidence of 0.5-2.0 new cases per million population per year, approximately 30-50% of all cases represent childhood craniopharyngioma. Therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining functions of the optical nerve and hypothalamic-pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but relapses and reduced QoL in survivors are also frequent. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007). Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and QoL consequences in order to provide optimal care of surviving patients.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Child
  • Craniopharyngioma / diagnosis
  • Craniopharyngioma / mortality
  • Craniopharyngioma / therapy*
  • Humans
  • Treatment Outcome*