Papillary Craniopharyngioma: an integrative and comprehensive review.

Autor:	Prieto R; Department of Neurosurgery, Puerta de Hierro University Hospital, Madrid, Spain., Juratli TA; Department of Neurosurgery, Laboratory of Translational Neuro-Oncology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, United States.; Department of Neurosurgery, Division of Neuro-Oncology, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.; National Center for Tumor Diseases (NCT), Partner Site Dresden, Dresden, Germany., Bander ED; Department of Neurosurgery, MD Anderson Cancer Center, Houston, Texas, United States., Santagata S; Department of Pathology, Brigham and Women's Hospital, Boston, United States.; Department of Systems Biology, Boston, United States.; Laboratory of Systems Pharmacology, Harvard Medical School, Boston, United States., Barrios L; Department of Applied Statistics, SGAI-CSIC, Spanish National Research Council, Madrid, Spain., Brastianos PK; Department of Medicine, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA, United States., Schwartz TH; Department of Neurosurgery, New York, United States.; Department of Otolaryngology, New York, United States.; Department of Neuroscience, Weill Cornell Medical College, New York Presbyterian Hospital, New York, United States., Pascual JM; Department of Neurosurgery, La Princesa University Hospital, Madrid, Spain.
Jazyk:	angličtina
Zdroj:	Endocrine reviews [Endocr Rev] 2024 Oct 01. Date of Electronic Publication: 2024 Oct 01.
DOI:	10.1210/endrev/bnae028
Abstrakt:	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 type. 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. (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. See the journal About page for additional terms.)
Databáze:	MEDLINE
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