{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T05:29:25Z","timestamp":1786771765959,"version":"build-2736575974"},"reference-count":32,"publisher":"Japan Neurosurgical Society","issue":"0","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,12,31]]},"DOI":"10.2176\/jns-nmc.2026-0094","type":"journal-article","created":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T22:10:33Z","timestamp":1786659033000},"page":"393-399","source":"Crossref","is-referenced-by-count":0,"title":["Leptomeningeal Dissemination in TERT Promoter-mutant Anaplastic Pleomorphic Xanthoastrocytoma Responding to BRAF\u2013MEK Inhibition: A Case Report"],"prefix":"10.2176","volume":"13","author":[{"given":"Ryota","family":"KITANO","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Kobe University Graduate School of Medicine"},{"name":"Department of Neurosurgery, Kobe City Nishi-Kobe Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"NAGASHIMA","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe University Graduate School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"GOTO","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe University Graduate School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"YAMANISHI","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe City Nishi-Kobe Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masamitsu","family":"NISHIHARA","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe City Nishi-Kobe Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiro","family":"TANAKA","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe University Graduate School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"MURAGAKI","sequence":"additional","affiliation":[{"name":"Center for Advanced Medical Engineering Research and Development, Kobe University"},{"name":"Department of Neurosurgery, Tokyo Women&apos;s Medical University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"SASAYAMA","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Kobe University Graduate School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"971","reference":[{"key":"1","doi-asserted-by":"publisher","unstructured":"1) Ostrom QT, Cioffi G, Gittleman H, et al. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2012-2016. Neuro Oncol. 2019;21 Suppl 5:v1-100. doi: 10.1093\/neuonc\/noz150","DOI":"10.1093\/neuonc\/noz150"},{"key":"2","doi-asserted-by":"publisher","unstructured":"2) Shaikh N, Brahmbhatt N, Kruser TJ, et al. Pleomorphic xanthoastrocytoma: a brief review. CNS Oncol. 2019;8 (3):CNS39. doi: 10.2217\/cns-2019-0009","DOI":"10.2217\/cns-2019-0009"},{"key":"3","doi-asserted-by":"publisher","unstructured":"3) Rutkowski MJ, Oh T, Niflioglu GG, et al. Pleomorphic xanthoastrocytoma with anaplastic features: retrospective case series. World Neurosurg. 2016;95:368-74. doi: 10.1016\/j.wneu.2016.07.068","DOI":"10.1016\/j.wneu.2016.07.068"},{"key":"4","doi-asserted-by":"publisher","unstructured":"4) Zuo P, Li T, Sun T, et al. Clinical features and surgical outcomes of high grade pleomorphic xanthoastrocytomas: a single-center experience with a systematic review. Front Oncol. 2023;13:1193611. doi: 10.3389\/fonc.2023.1193611","DOI":"10.3389\/fonc.2023.1193611"},{"key":"5","doi-asserted-by":"publisher","unstructured":"5) Phillips JJ, Gong H, Chen K, et al. The genetic landscape of anaplastic pleomorphic xanthoastrocytoma. Brain Pathol. 2019;29 (1):85-96. doi: 10.1111\/bpa.12639","DOI":"10.1111\/bpa.12639"},{"key":"6","doi-asserted-by":"publisher","unstructured":"6) Vaubel R, Zschernack V, Tran QT, et al. Biology and grading of pleomorphic xanthoastrocytoma-what have we learned about it? Brain Pathol. 2021;31 (1):20-32. doi: 10.1111\/bpa.12874","DOI":"10.1111\/bpa.12874"},{"key":"7","doi-asserted-by":"publisher","unstructured":"7) Andrews LJ, Thornton ZA, Saincher SS, et al. Prevalence of BRAF V600 in glioma and use of BRAF Inhibitors in patients with BRAF V600 mutation-positive glioma: systematic review. Neuro Oncol. 2022;24 (4):528-40. doi: 10.1093\/neuonc\/noab247","DOI":"10.1093\/neuonc\/noab247"},{"key":"8","doi-asserted-by":"publisher","unstructured":"8) Ma C, Feng R, Chen H, et al. BRAF v600e, TERT, and IDH2 mutations in pleomorphic xanthoastrocytoma: observations from a large case-series study. World Neurosurg. 2018;120:e1225-33. doi: 10.1016\/j.wneu.2018.09.050","DOI":"10.1016\/j.wneu.2018.09.050"},{"key":"9","doi-asserted-by":"publisher","unstructured":"9) Andersen BM, Miranda C, Hatzoglou V, et al. Leptomeningeal metastases in glioma: the Memorial Sloan Kettering Cancer Center experience. Neurology. 2019;92 (21):e2483. doi: 10.1212\/WNL.0000000000007529","DOI":"10.1212\/WNL.0000000000007529"},{"key":"10","doi-asserted-by":"publisher","unstructured":"10) Berzero G, Bellu L, Baldini C, et al. Sustained tumor control with MAPK inhibition in BRAF v600-mutant adult glial and glioneuronal tumors. Neurology. 2021;97 (7):e673-e83. doi: 10.1212\/WNL.0000000000012330","DOI":"10.1212\/WNL.0000000000012330"},{"key":"11","doi-asserted-by":"publisher","unstructured":"11) Wen PY, Stein A, van den Bent M, et al. Dabrafenib plus trametinib in patients with BRAFV600E-mutant low-grade and high-grade glioma (ROAR): a multicentre, open-label, single-arm, phase 2, basket trial. Lancet Oncol. 2022;23 (1):53-64. doi: 10.1016\/S1470-2045(21)00578-7","DOI":"10.1016\/S1470-2045(21)00578-7"},{"key":"12","doi-asserted-by":"publisher","unstructured":"12) Kepes JJ. Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. Brain Pathol. 1993;3 (3):269-74. doi: 10.1111\/j.1750-3639.1993.tb00753.x","DOI":"10.1111\/j.1750-3639.1993.tb00753.x"},{"key":"13","doi-asserted-by":"publisher","unstructured":"13) Vaubel RA, Caron AA, Yamada S, et al. Recurrent copy number alterations in low-grade and anaplastic pleomorphic xanthoastrocytoma with and without BRAF v600e mutation. Brain Pathol. 2018;28 (2):172-82. doi: 10.1111\/bpa.12495","DOI":"10.1111\/bpa.12495"},{"key":"14","doi-asserted-by":"publisher","unstructured":"14) Olympios N, Gilard V, Marguet F, et al. Tert promoter alterations in glioblastoma: a systematic review. Cancers. 2021;13 (5):1147. doi: 10.3390\/cancers13051147","DOI":"10.3390\/cancers13051147"},{"key":"15","doi-asserted-by":"publisher","unstructured":"15) Lee EQ, Ruland S, LeBoeuf NR, et al. Successful treatment of a progressive BRAF v600e-mutated anaplastic pleomorphic xanthoastrocytoma with vemurafenib monotherapy. J Clin Oncol. 2016;34 (10):e87-9. doi: 10.1200\/JCO.2013.51.1766","DOI":"10.1200\/JCO.2013.51.1766"},{"key":"16","doi-asserted-by":"publisher","unstructured":"16) Burger MC, Ronellenfitsch MW, Lorenz NI, et al. Dabrafenib in patients with recurrent, BRAF v600e mutated malignant glioma and leptomeningeal disease. Oncol Rep. 2017;38 (6):3291-6. doi: 10.3892\/or.2017.6013","DOI":"10.3892\/or.2017.6013"},{"key":"17","doi-asserted-by":"publisher","unstructured":"17) Amayiri N, Swaidan M, Al-Hussaini M, et al. Sustained response to targeted therapy in a patient with disseminated anaplastic pleomorphic xanthoastrocytoma. J Pediatr Hematol Oncol. 2018;40 (6):478-82. doi: 10.1097\/MPH.0000000000001032","DOI":"10.1097\/MPH.0000000000001032"},{"key":"18","doi-asserted-by":"publisher","unstructured":"18) Smith-Cohn M, Davidson C, Colman H, et al. Challenges of targeting BRAF V600E mutations in adult primary brain tumor patients: a report of two cases. CNS Oncol. 2019;8 (4):CNS48. doi: 10.2217\/cns-2019-0018","DOI":"10.2217\/cns-2019-0018"},{"key":"19","doi-asserted-by":"publisher","unstructured":"19) Thomas AA, Tucker SM, Nelson CJ, et al. Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab. Pediatr Blood Cancer. 2019;66 (1):e27465. doi: 10.1002\/pbc.27465","DOI":"10.1002\/pbc.27465"},{"key":"20","doi-asserted-by":"publisher","unstructured":"20) Pi\u00f1a Y, Fusco MJ, Macaulay RJ, et al. Using personalized medicine in gliomas: A genomic approach to diagnosis and overcoming treatment resistance in a case with pleomorphic xanthoastrocytoma. J Neurol. 2020;267 (3):783-90. doi: 10.1007\/s00415-019-09575-8","DOI":"10.1007\/s00415-019-09575-8"},{"key":"21","doi-asserted-by":"publisher","unstructured":"21) Kata K, Rodriguez-Quintero JC, Arevalo OD, et al. BRAF\/MEK dual inhibitors therapy in progressive and anaplastic pleomorphic xanthoastrocytoma: case series and literature review. J Natl Compr Canc Netw. 2022;20 (11):1193-1202.e6. doi: 10.6004\/jnccn.2022.7046","DOI":"10.6004\/jnccn.2022.7046"},{"key":"22","doi-asserted-by":"publisher","unstructured":"22) Bazer DA, Kolchinski A, Bush NAO, et al. No drug holidays in BRAFV600E glioma patients: an argument for dose reduction of targeted therapies. Neurooncol Pract. 2024;11 (5):660-4. doi: 10.1093\/nop\/npae046","DOI":"10.1093\/nop\/npae046"},{"key":"23","doi-asserted-by":"publisher","unstructured":"23) Castelli B, Tellini M, Guidi M, et al. Case report: complete long-lasting response to multimodal third line treatment with neurosurgical resection, carmustine wafer implantation and dabrafenib plus trametinib in a BRAFV600E mutated high-grade glioma. Front Oncol. 2024;14:1359093. doi: 10.3389\/fonc.2024.1359093","DOI":"10.3389\/fonc.2024.1359093"},{"key":"24","doi-asserted-by":"crossref","unstructured":"24) Inoue H, Kuroda JI, Fujioka Y, et al. Drug-resistant BRAF V600E-mutant recurrent pleomorphic xanthoastrocytoma, cns who grade 3 successfully resolved with incidental discontinuation of combined BRAF and MEK inhibitor therapy. Surg Neurol Int. 2024;15:417. doi: 10.25259\/SNI_734_2024","DOI":"10.25259\/SNI_734_2024"},{"key":"25","doi-asserted-by":"publisher","unstructured":"25) Kawaguchi Y, Watanabe Y, Miyakita Y, et al. Successful treatment of pediatric patients with high-grade gliomas featuring leptomeningeal metastases by targeting BRAF V600E mutations with dabrafenib plus trametinib: two illustrative cases. Int Cancer Conf J. 2024;13 (3):256-62. doi: 10.1007\/s13691-024-00674-6","DOI":"10.1007\/s13691-024-00674-6"},{"key":"26","doi-asserted-by":"publisher","unstructured":"26) Vermeulen E, Vander Mijnsbrugge AS, Brock S, et al. Successful treatment with dabrafenib\/trametinib of a malignantly transformed and metastasized BRAF V600E mutant pleiomorphic xanthoastrocytoma: a case report and review of the literature. Case Rep Oncol. 2024;17 (1):10-6. doi: 10.1159\/000534731","DOI":"10.1159\/000534731"},{"key":"27","doi-asserted-by":"publisher","unstructured":"27) Chamberlain MC. Salvage therapy with BRAF inhibitors for recurrent pleomorphic xanthoastrocytoma: a retrospective case series. J Neurooncol. 2013;114 (2):237-40. doi: 10.1007\/s11060-013-1176-5","DOI":"10.1007\/s11060-013-1176-5"},{"key":"28","doi-asserted-by":"publisher","unstructured":"28) Usubalieva A, Pierson CR, Kavran CA, et al. Primary meningeal pleomorphic xanthoastrocytoma with anaplastic features: a report of 2 cases, one with BRAF V600E mutation and clinical response to the BRAF inhibitor. J Neuropathol Exp Neurol. 2015;74 (10):960-9. doi: 10.1097\/Nen.0000000000000240","DOI":"10.1097\/NEN.0000000000000240"},{"key":"29","doi-asserted-by":"crossref","unstructured":"29) Tan J, Liu R, Zhu G, et al. Tert promoter mutation determines apoptotic and therapeutic responses of BRAF-mutant cancers to BRAF and MEK inhibitors: Achilles heel. Proc Natl Acad Sci U S A. 2020;117 (27):15846-51. doi: 10.1073\/pnas.2004707117","DOI":"10.1073\/pnas.2004707117"},{"key":"30","doi-asserted-by":"publisher","unstructured":"30) Thielmann CM, Matull J, Zaremba A, et al. TERT promoter mutations are associated with longer progression-free and overall survival in patients with BRAF-mutant melanoma receiving BRAF and MEK inhibitor therapy. Eur J Cancer. 2022;161:99-107. doi: 10.1016\/j.ejca.2021.11.009","DOI":"10.1016\/j.ejca.2021.11.009"},{"key":"31","unstructured":"31) WHO Classification of Tumours Editorial Board. Central nervous system tumours: who classification of tumours. Lyon, France: International Agency for Research on Cancer; 2021. 608 p."},{"key":"32","doi-asserted-by":"publisher","unstructured":"32) Reinhardt A, Stichel D, Schrimpf D, et al. Anaplastic astrocytoma with piloid features, a novel molecular class of IDH wildtype glioma with recurrent mapk pathway, cdkn2a\/b and ATRX alterations. Acta Neuropathol. 2018;136 (2):273-91. doi: 10.1007\/s00401-018-1837-8","DOI":"10.1007\/s00401-018-1837-8"}],"container-title":["NMC Case Report Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/nmccrj\/13\/0\/13_2026-0094\/_pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T05:23:05Z","timestamp":1786771385000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/nmccrj\/13\/0\/13_2026-0094\/_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,12,31]]},"references-count":32,"journal-issue":{"issue":"0","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.2176\/jns-nmc.2026-0094","relation":{},"ISSN":["2188-4226"],"issn-type":[{"value":"2188-4226","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,12,31]]},"article-number":"2026-0094"}}