Article ; Online: Childhood-onset Craniopharyngioma.
The Journal of clinical endocrinology and metabolism
2021 Volume 106, Issue 10, Page(s) e3820–e3836
Abstract: 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 ... ...
| Abstract | 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 "craniopharyngioma, hypothalamus, pituitary obesity, irradiation, neurosurgery. |
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| MeSH term(s) | Age of Onset ; Child ; Comorbidity ; Craniopharyngioma/diagnosis ; Craniopharyngioma/epidemiology ; Craniopharyngioma/therapy ; History, 20th Century ; History, 21st Century ; Humans ; Neurosurgical Procedures/history ; Neurosurgical Procedures/methods ; Neurosurgical Procedures/trends ; Pituitary Neoplasms/diagnosis ; Pituitary Neoplasms/epidemiology ; Pituitary Neoplasms/therapy ; Quality of Life ; Survival Rate |
| Language | English |
| Publishing date | 2021-11-30 |
| Publishing country | United States |
| Document type | Historical Article ; Journal Article ; Research Support, Non-U.S. Gov't ; Review |
| ZDB-ID | 3029-6 |
| ISSN | 1945-7197 ; 0021-972X |
| ISSN (online) | 1945-7197 |
| ISSN | 0021-972X |
| DOI | 10.1210/clinem/dgab397 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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