Papillary Craniopharyngioma [Neurosurgery Wiki]

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====== Papillary Craniopharyngioma ======

//[[J.Sales-Llopis]]//

//Neurosurgery Department, [[General University Hospital Alicante]], [[Spain]]//
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{{rss>https://pubmed.ncbi.nlm.nih.gov/rss/search/1zCzv8cgXYLQFq_OldqCAHHRNLwgCPb24UfXWrfqOAyoXkhE95/?limit=15&utm_campaign=pubmed-2&fc=20241004025341}}
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Papillary [[craniopharyngioma]] (PCP) is a rare type of tumor, comprising ∼20% of all craniopharyngioma (CP) cases. It is now recognized as a separate pathological entity from the [[adamantinomatous craniopharyngioma]] type. 

PCPs are different from ACPs regards the clinical features, operative techniques and outcomes. If necessary, PCPs are suggested more amenable to total removal since its less invasiveness to the 3rd V floor and better hypothalamic outcomes
((Yang L, Liu Y, Wang C, Feng Z, Yu L, Pan J, Peng J, Nie J, Zhou M, Ou Y, Liu T, Qi S, Fan J. Distinction of papillary and adamantinomatous craniopharyngioma: Clinical features, surgical nuances and hypothalamic outcomes. Neoplasia. 2024 Oct 1;57:101060. doi: 10.1016/j.neo.2024.101060. Epub ahead of print. PMID: 39357265.))
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PCPs are benign tumors, classified as WHO grade 1, characterized by non-keratinizing squamous epithelium. They typically grow as solid and round papillomatous masses or as unilocular cysts with a cauliflower-like excrescence. PCPs primarily occur in adults (95%), with increased frequency in males (60%) and predominantly affect the hypothalamus. Over 80% of these tumors are located in the third ventricle, expanding either above an anatomically intact infundibulum (strictly third ventricle tumors) or within the infundibulo-tuberal region of the third ventricle floor. Clinical manifestations commonly include visual deficits and a wide range of psychiatric disturbances (45% of patients), such as memory deficits and odd behavior. MRI can identify up to 50% of PCPs by the presence of a basal duct-like recess. Surgical management is challenging, requiring complex approaches to the third ventricle and posing significant risk of hypothalamic injury. The endoscopic endonasal approach allows radical tumor resection and yields more favorable patient outcomes. Of intriguing pathogenesis, over 90% of PCPs harbor the somatic BRAFV600E mutation, which activates the mitogen-activated protein kinase (MAPK/ERK) signaling pathway. A phase 2 clinical trial has demonstrated that PCPs respond well to BRAF/MEK inhibitors. This comprehensive review synthesizes information from a cohort of 560 well-described PCPs and 99 large CP series including PCP cases published from 1856-2023 and represents the most extensive collection of knowledge on PCPs to date
((Prieto R, Juratli TA, Bander ED, Santagata S, Barrios L, Brastianos PK, Schwartz TH, Pascual JM. Papillary Craniopharyngioma: an integrative and comprehensive review. Endocr Rev. 2024 Oct 1:bnae028. doi: 10.1210/endrev/bnae028. Epub ahead of print. PMID: 39353067.))