{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T06:06:32Z","timestamp":1784009192742,"version":"3.55.0"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"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":[[2026,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Rathke cleft cysts are benign sellar lesions with a substantial risk of postoperative recurrence. In contrast to papillary craniopharyngiomas, oncogenic BRAF mutations are exceptionally reported in Rathke cleft cysts (RCCs), and the therapeutic relevance of targeted inhibition remains unknown.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>A 51-y-old man presented with a giant cystic suprasellar lesion involving the optochiasmatic and subfrontal regions. Following incomplete surgical resection, histopathological analysis confirmed the diagnosis of Rathke\u2019s cleft cyst. Molecular profiling identified a somatic BRAF p. V600E mutation present in a subclonal population. Rapid recurrence occurred 3 months postoperatively. Combined BRAF\/MEK inhibition with dabrafenib and trametinib was initiated, leading to a marked reduction in cyst volume on serial MRI, further enhanced after dose escalation. Treatment was well tolerated, with complete symptom resolution and no endocrine impairment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This report describes the first adult BRAF-mutant RCC responding to BRAF\/MEK-targeted therapy, supporting molecular profiling to guide personalized management in recurrent or high-risk RCCs.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/nop\/npag035","type":"journal-article","created":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T11:42:28Z","timestamp":1776339748000},"page":"823-827","source":"Crossref","is-referenced-by-count":0,"title":["BRAF\u2013MEK inhibition in a giant BRAF-mutant Rathke cleft cyst: Diagnostic challenges and clinical implications for BRAF 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Croissance et du D\u00e9veloppement, CRMERC, Endo-ERN, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, AP-HP, Sorbonne University , Paris,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Coulet","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Sorbonne Universit\u00e9, Groupe Hospitalier Piti\u00e9-Salp\u00eatri\u00e8re, APHP , Paris,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne","family":"Tran","sequence":"additional","affiliation":[{"name":"Department of Pathology, Assistance Publique-H\u00f4pitaux de Paris, Lariboisi\u00e8re University Hospital , Paris,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5290-1204","authenticated-orcid":false,"given":"Ahmed","family":"Idbaih","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, AP-HP, Institut du Cerveau\u2014Paris Brain 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