{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T03:51:21Z","timestamp":1793332281027,"version":"4.5.0"},"reference-count":184,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2021,6,4]],"date-time":"2021-06-04T00:00:00Z","timestamp":1622764800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/501100007311","name":"German Childhood Cancer Foundation","doi-asserted-by":"publisher","award":["DKS2014.13"],"award-info":[{"award-number":["DKS2014.13"]}],"id":[{"id":"10.13039\/501100007311","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,9,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Craniopharyngiomas are rare embryonic malformational tumors of the sellar\/parasellar region, classified by the World Health Organization (WHO) as tumors with low-grade malignancy (WHO I). The childhood adamantinomatous subtype of craniopharyngioma is usually cystic with calcified areas. At the time of diagnosis, hypothalamic\/pituitary deficits, visual disturbances, and increased intracranial pressure are major symptoms. The treatment of choice in case of favorable tumor location (without hypothalamic involvement) is complete resection. It is important to ensure that optical and hypothalamic functionality are preserved. In case of unfavorable tumor location, that is with hypothalamic involvement, a hypothalamus-sparing surgical strategy with subsequent local irradiation of residual tumor is recommended. In the further course of the disease, recurrences and progression often occur. Nevertheless, overall survival rates are high at 92%. Severe impairment of quality of life and comorbidities such as metabolic syndrome, hypothalamic obesity, and neurological consequences can be observed in patients with disease- and\/or treatment-related lesions of hypothalamic structures. Childhood-onset craniopharyngioma frequently manifests as a chronic disease so that patients require lifelong, continuous care by experienced multidisciplinary teams to manage clinical and quality of life consequences. For this review, a search for original articles and reviews published between 1986 and 2020 was performed in Pubmed, Science Citation Index Expanded, EMBASE, and Scopus. The search terms used were \u201ccraniopharyngioma, hypothalamus, pituitary obesity, irradiation, neurosurgery.<\/jats:p>","DOI":"10.1210\/clinem\/dgab397","type":"journal-article","created":{"date-parts":[[2021,6,4]],"date-time":"2021-06-04T16:10:16Z","timestamp":1622823016000},"page":"e3820-e3836","source":"Crossref","is-referenced-by-count":68,"title":["Childhood-onset Craniopharyngioma"],"prefix":"10.1210","volume":"106","author":[{"given":"Anna","family":"Otte","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Pediatric Hematology\/Oncology, University Children\u00b4s Hospital, Carl von Ossietzky University Oldenburg, Klinikum Oldenburg A\u00f6R, 26133 Oldenburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4929-9966","authenticated-orcid":false,"given":"Hermann L","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Pediatric Hematology\/Oncology, University Children\u00b4s Hospital, Carl von Ossietzky University Oldenburg, Klinikum Oldenburg A\u00f6R, 26133 Oldenburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2021,6,4]]},"reference":[{"issue":"6","key":"2021110611595054500_CIT0001","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1007\/s00401-016-1545-1","article-title":"The 2016 World Health Organization classification of tumors of the central nervous system: a summary","volume":"131","author":"Louis","year":"2016","journal-title":"Acta Neuropathol."},{"issue":"4","key":"2021110611595054500_CIT0002","doi-asserted-by":"crossref","first-page":"547","DOI":"10.3171\/jns.1998.89.4.0547","article-title":"The descriptive epidemiology of craniopharyngioma","volume":"89","author":"Bunin","year":"1998","journal-title":"J Neurosurg."},{"issue":"1","key":"2021110611595054500_CIT0003","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1530\/EJE-11-0158","article-title":"Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after 3-year follow-up","volume":"165","author":"M\u00fcller","year":"2011","journal-title":"Eur J Endocrinol."},{"issue":"2","key":"2021110611595054500_CIT0004","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1210\/jc.2014-3525","article-title":"Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden","volume":"100","author":"Olsson","year":"2015","journal-title":"J Clin Endocrinol Metab."},{"issue":"8","key":"2021110611595054500_CIT0005","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1093\/neuonc\/nos142","article-title":"Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program","volume":"14","author":"Zacharia","year":"2012","journal-title":"Neuro Oncol."},{"issue":"3","key":"2021110611595054500_CIT0006","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1007\/s11060-011-0540-6","article-title":"Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults","volume":"104","author":"Nielsen","year":"2011","journal-title":"J Neurooncol."},{"issue":"2","key":"2021110611595054500_CIT0007","doi-asserted-by":"crossref","first-page":"206","DOI":"10.3171\/jns.1995.83.2.0206","article-title":"Papillary craniopharyngioma: a clinicopathological study of 48 cases","volume":"83","author":"Crotty","year":"1995","journal-title":"J Neurosurg."},{"issue":"10","key":"2021110611595054500_CIT0008","doi-asserted-by":"crossref","first-page":"689","DOI":"10.2176\/nmc.51.689","article-title":"Xanthogranuloma of the sellar region of children: series of five cases and literature review","volume":"51","author":"Kamoshima","year":"2011","journal-title":"Neurol Med Chir (Tokyo)."},{"issue":"3-4","key":"2021110611595054500_CIT0009","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1007\/BF01401226","article-title":"Craniopharyngioma in Finland. 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