Craniopharyngioma
Top Cited Papers
- 27 January 2014
- journal article
- review article
- Published by The Endocrine Society in Endocrine Reviews
- Vol. 35 (3), 513-543
- https://doi.org/10.1210/er.2013-1115
Abstract
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.Keywords
This publication has 256 references indexed in Scilit:
- Identification of novel pathways involved in the pathogenesis of human adamantinomatous craniopharyngiomaActa Neuropathologica, 2012
- On the Benefits and Risks of Proton Therapy in Pediatric CraniopharyngiomaInternational Journal of Radiation Oncology*Biology*Physics, 2012
- Surgical Outcome in 90 Patients with Craniopharyngioma: An Evaluation of Transsphenoidal SurgeryWorld Neurosurgery, 2010
- Childhood craniopharyngioma—current concepts in diagnosis, therapy and follow-upNature Reviews Endocrinology, 2010
- The role of fractionated radiotherapy and radiosurgery in the management of patients with craniopharyngiomaNeurosurgical Review, 2009
- Gastric bypass surgery for treatment of hypothalamic obesity after craniopharyngioma therapyNature Clinical Practice Endocrinology & Metabolism, 2007
- Melatonin Treatment in Obese Patients with Childhood Craniopharyngioma and Increased Daytime SleepinessCancer Causes & Control, 2006
- Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngiomaThe Journal of Pathology, 2004
- Prenatal diagnosis of a craniopharyngioma using ultrasonography and magnetic resonance imagingPrenatal Diagnosis, 1990
- Microsurgery of tumours of diencephalic regionNeurosurgical Review, 1983