{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T17:20:46Z","timestamp":1792171246269,"version":"4.4.0"},"reference-count":437,"publisher":"The Endocrine Society","issue":"2","license":[{"start":{"date-parts":[[2024,10,1]],"date-time":"2024-10-01T00:00:00Z","timestamp":1727740800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Papillary craniopharyngioma (PCP) is a rare type of tumor, comprising \u223c20% of all craniopharyngioma (CP) cases. It is now recognized as a separate pathological entity from the adamantinomatous type. PCPs are benign tumors, classified as World Health Organization grade 1, characterized by nonkeratinizing 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. Magnetic resonance imaging 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 signaling pathway. A phase 2 clinical trial has demonstrated that PCPs respond well to proto-oncogene B-Raf\/MAPK\/ERK kinase 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 to 2023 and represents the most extensive collection of knowledge on PCPs to date.<\/jats:p>","DOI":"10.1210\/endrev\/bnae028","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T07:18:30Z","timestamp":1727680710000},"page":"151-213","source":"Crossref","is-referenced-by-count":17,"title":["Papillary Craniopharyngioma: An Integrative and Comprehensive Review"],"prefix":"10.1210","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1401-1224","authenticated-orcid":false,"given":"Ruth","family":"Prieto","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Puerta de Hierro University Hospital , 28222 Madrid ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tareq A","family":"Juratli","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Laboratory of Translational Neuro-Oncology, Massachusetts General Hospital Cancer Center, Harvard Medical School , Boston, MA 02114 ,","place":["USA"]},{"name":"Department of Neurosurgery, Division of Neuro-Oncology, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden ,","place":["Germany"]},{"name":"National Center for Tumor Diseases, Partner Site Dresden , 01307 Dresden ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evan D","family":"Bander","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, MD Anderson Cancer Center , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandro","family":"Santagata","sequence":"additional","affiliation":[{"name":"Department of Pathology, Brigham and Women's Hospital, Harvard Medical School , Boston, MA 02115 ,","place":["USA"]},{"name":"Department of Systems Biology, Harvard Medical School , Boston, MA 02115 ,","place":["USA"]},{"name":"Laboratory of Systems Pharmacology, Harvard Medical School , Boston, MA 02115 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Barrios","sequence":"additional","affiliation":[{"name":"Department of Applied Statistics, SGAI-CSIC, Spanish National Research Council , 28002 Madrid ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priscilla K","family":"Brastianos","sequence":"additional","affiliation":[{"name":"Department of Medicine, Massachusetts General Hospital Cancer Center, Harvard Medical School , Boston, MA 02114 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4464-7394","authenticated-orcid":false,"given":"Theodore H","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Weill Cornell Medical College, New York Presbyterian Hospital , New York, NY 10065 ,","place":["USA"]},{"name":"Department of Otolaryngology, Weill Cornell Medical College, New York Presbyterian Hospital , New York, NY 10065 ,","place":["USA"]},{"name":"Department of Neuroscience, Weill Cornell Medical College, New York Presbyterian Hospital , New York, NY 10065 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3114-0474","authenticated-orcid":false,"given":"Jos\u00e9 M","family":"Pascual","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, La Princesa University Hospital , 28006 Madrid 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