Endocr Rev. 2014 Jun;35(3):513-43. doi: 10.1210/er.2013-1115. Epub 2014 Jan 27.


This report is a review of findings on the diagnosis, treatment, clinical course, and prognosis of craniopharyngioma patients. Craniopharyngiomas are rare, partly cystic and calcified embryonic malformations of the sellar/parasellar region with low histological grade (WHO I°). A bimodal age distribution has been shown, with peak incidence rates in childhood-onset at 5-14 years and adult-onset craniopharyngioma at 50-74 years. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Although overall survival rates are high (92%), recurrences and progressions are frequent. Irradiation has proven effective in reducing recurrences and progression, and timing of postsurgical irradiation in childhood-onset cases is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious quality of life-compromising sequelae such as hypothalamic obesity, psychopathological symptoms, and/or cognitive problems. It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and quality of life consequences by experienced multidisciplinary teams.

Publication types

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

MeSH terms

  • Craniopharyngioma* / diagnosis
  • Craniopharyngioma* / epidemiology
  • Craniopharyngioma* / therapy
  • Humans
  • Pituitary Neoplasms* / diagnosis
  • Pituitary Neoplasms* / epidemiology
  • Pituitary Neoplasms* / therapy