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- Title
Czaszkogardlak - epidemiologia, patogeneza, diagnostyka klinicznych i obrazowa.
- Authors
Moszczyńska, Elżbieta; Szalecki, Mieczysław; Pasternak-Pietrzak, Katarzyna; Jurkiewicz, Elżbieta; Szymańska, Sylwia
- Abstract
Craniopharyngioma is a slowly growing, histologically benign tumor of the sellar region (I grade in WHO classification), there have been described rare cases of malignant transformation. It contributes to 5,6-14,1% of brain tumors in children. Incidence rate is 1,3-2,1/million/year and around 30-50% are diagnosed in children even as early as in their prenatal and neonatal period. There are 2 hypothesis of tumor etiopathology: the tumor origins from ectodermal remnants of Rathke's pouch (ameloblasticum type) or it arises from metaplasia of residual embryonal epithelium of the anterior pituitary gland and of the anterior infundibulum (papillary type). Adamantinomatous type is more frequent in children and papillary type dominates in adults. In around 50-95% of cases of adamantinomatous type an activating mutation in the gene CTNN1encoding ß-catenin is present. BRAF V600E mutation is a genetic background of papillary craniopharyngioma, present in 95% of patients. The method of choice for craniopharyngioma diagnostics is MR imaging with contrast agents. The most frequent symptoms of craniopharyngioma are: increased intracranial pressure, visual disturbanobces and hypopituitarism. An analysis of risk factors of craniopharyngioma recurrence.
- Publication
Pediatric Endocrinology / Endokrynologia Pediatryczna, 2017, Vol 16, Issue 3, p205
- ISSN
1730-0282
- Publication type
Article
- DOI
10.18544/EP-01.16.03.1676