{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T13:21:41Z","timestamp":1793020901416,"version":"4.5.0"},"reference-count":39,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T00:00:00Z","timestamp":1786665600000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurooncol"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Targeted therapy for BRAF V600E mutant papillary craniopharyngioma (PCP) has been rapidly accepted, though given the rarity of the tumor, there is limited guidance for appropriate use. Here, we retrospectively evaluated current practice patterns for the implementation of targeted therapeutics and their outcomes in the treatment of papillary craniopharyngioma (PCP).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Practice patterns at the institutions in the Registry for Adenomas of the Pituitary and Related Disorders (RAPID) for treatment of BRAF V600E mutated PCPs using targeted therapy were evaluated. Baseline clinical and demographic variables were retrospectively recorded. Imaging response was evaluated. Dosing, treatment course, adverse events (AEs) and other therapeutic strategies employing BRAF\/MEK inhibitors were studied. Adjuvant and\/or salvage therapies, including radiation and surgery were recorded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Most patients demonstrated at least partial response to BRAF and\/or BRAF\/MEK inhibition (13\/19, 72.2%). No patients progressed through therapy. Grade 0\u20132 AEs occurred in 79% of cases, and nine (47.4%) patients eventually discontinued therapy. Radiation-sparing regimens demonstrated similar radiographic response rates and had smaller residual tumor volume after treatment completion (23.7 vs. 612.4 mm\n                      <jats:sup>3<\/jats:sup>\n                      ,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.040) compared to those receiving radiation. BRAF monotherapy demonstrated similar rates of response to therapy, but fewer Grade 3\u20134 AEs (0% vs. 28.5%) than dual therapy. Long-course therapy (&gt;\u20094 cycles) resulted in radiographic response (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.024) more often than a short-course (\u2264\u20094 cycles), with similar AEs. Median follow-up duration was 19 months from surgery (range: 8\u201363).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Critical insights into molecular therapeutic practice patterns for treatment of PCP were identified. Radiation-sparing, mono-therapeutic, and prolonged treatment duration are feasible options and warrant further study.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11060-026-05739-5","type":"journal-article","created":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T09:09:41Z","timestamp":1786698581000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Molecular therapy for papillary craniopharyngioma: a multi-institutional analysis of practice patterns across the RAPID Consortium"],"prefix":"10.1007","volume":"179","author":[{"given":"Mark A.","family":"Damante","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan B.","family":"Juncker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew S.","family":"Little","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie J.","family":"Van Gompel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandhya","family":"Palit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donato R.","family":"Pacione","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garni","family":"Barkhoudarian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walavan","family":"Sivakumar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Spiros","family":"Blackburn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle Maga\u00f1a","family":"Mendoza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James J.","family":"Evans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Benjamin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua D.","family":"Palmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Giglio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel M.","family":"Prevedello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle C.","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"RAPID Consortium","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,8,14]]},"reference":[{"issue":"1","key":"5739_CR1","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1038\/s41572-019-0125-9","volume":"5","author":"HL M\u00fcller","year":"2019","unstructured":"M\u00fcller HL, Merchant TE, Warmuth-Metz M, Martinez-Barbera JP, Puget S (2019) Craniopharyngioma, (in eng). 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Approval was granted by the ethics committee at The Ohio State University Biomedical Institutional Review Board (#2022H0179). The overarching multi-institutional IRB oversight was provided by the Institutional Review Board at Barrow Neurological Institute.","order":1,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"J.J.E. is a consultant for and has received royalties from Mizuho. G.B. has received consulting fees from Cerevasc Inc., SpiWay Inc., and Zeiss Corp. J.V.G. is a named inventor on intellectual property licensed to Cadence Neuroscience Inc. D.M.P. has received consulting fees from Leica, Stryker, BK Medical, and KLS Martin. K.C.W. has received consulting fees from Retromer Therapeutics and acts as an advisor with stock ownership to Zeta Surgical. None of these disclosures apply or are relevant to the presented work. All remaining authors have no relevant financial or non-financial interests to disclose.","order":2,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"40"}}