{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T10:26:41Z","timestamp":1770114401902,"version":"3.49.0"},"reference-count":50,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"19","license":[{"start":{"date-parts":[[2025,5,12]],"date-time":"2025-05-12T00:00:00Z","timestamp":1747008000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Cosmet Laser Ther"],"published-print":{"date-parts":[[2025,5,12]]},"abstract":"\n BACKGROUND<\/jats:title>\n Anaplastic pleomorphic xanthoastrocytomas (APXAs) are rare, grade 3 malignant glial neoplasms first described as a distinct entity in the 2016 WHO classification of tumors of the CNS. They are generally seen in pediatric and young adult patients as supratentorial lesions with both solid and cystic components and have a high propensity for recurrence. APXAs occasionally demonstrate leptomeningeal dissemination (LMD) but very rarely at diagnosis.<\/jats:p>\n <\/jats:sec>\n \n OBSERVATIONS<\/jats:title>\n The authors describe the case of a 36-year-old male with a history of seizures and a known left temporal lesion since childhood, serially imaged and never biopsied, who presented with headache, increasingly frequent seizures, and right third nerve palsy. Imaging demonstrated significant interval enlargement of the lesion with intralesional hemorrhage and predominantly locoregional leptomeningeal enhancement. He underwent resection with gross-total resection achieved, and pathology revealed WHO grade 3 pleomorphic xanthoastrocytoma with ATG7::RAF1 fusion, 9p21 deletion, and telomerase reverse transcriptase promoter mutation, as identified by next-generation sequencing. This was followed by adjuvant whole-brain radiation therapy with boost and treatment with the MEK inhibitor cobimetinib.<\/jats:p>\n <\/jats:sec>\n \n LESSONS<\/jats:title>\n APXA, an already exceedingly rare primary CNS tumor, can present unusually in older adult patients with concomitant LMD and cranial neuropathy. Advanced genomic profiling can tailor adjuvant therapy.<\/jats:p>\n \n https:\/\/thejns.org\/doi\/10.3171\/CASE24768<\/jats:ext-link>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/case24768","type":"journal-article","created":{"date-parts":[[2025,5,12]],"date-time":"2025-05-12T20:15:30Z","timestamp":1747080930000},"source":"Crossref","is-referenced-by-count":0,"title":["Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination presenting with cranial neuropathy in an adult patient: illustrative case"],"prefix":"10.3171","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8226-1022","authenticated-orcid":true,"given":"Simon A.","family":"Menaker","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7485-0644","authenticated-orcid":true,"given":"Andre E.","family":"Boyke","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Serguei I.","family":"Bannykh","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ziedulla","family":"Abdullaev","sequence":"additional","affiliation":[{"name":"Departments of Laboratory of Pathology Clinical Methylation Unit, Center for Cancer Research, National Institutes of Health\/National Cancer Institute, Bethesda, Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lindsey B.","family":"Ross","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paula","family":"Eboli","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"issue":"6","key":"bib1","series-title":"Acta Neuropathol","first-page":"803","article-title":"The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary","volume":"131","author":"Louis","year":"2016","unstructured":"Louis DNPerry AReifenberger GThe 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131(6):803-820."},{"issue":"5","key":"bib2","series-title":"Brain Pathol","first-page":"575","article-title":"Pleomorphic xanthoastrocytoma: natural history and long-term follow-up","volume":"25","author":"Ida","year":"2015","unstructured":"Ida CMRodriguez FJBurger PCPleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol. 2015;25(5):575-586."},{"issue":"2","key":"bib3","series-title":"J Neurooncol","first-page":"343","article-title":"Impact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma","volume":"136","author":"Tonse","year":"2018","unstructured":"Tonse RGupta TEpari SImpact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma. J Neurooncol. 2018;136(2):343-350."},{"issue":"3","key":"bib4","series-title":"CNS Oncol","first-page":"CNS39","article-title":"Pleomorphic xanthoastrocytoma: a brief review","volume":"8","author":"Shaikh","year":"2019","unstructured":"Shaikh NBrahmbhatt NKruser TJPleomorphic xanthoastrocytoma: a brief review. CNS Oncol. 2019;8(3):CNS39."},{"issue":"4","key":"bib5","series-title":"Neuro Oncol","first-page":"446","article-title":"Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation","volume":"11","author":"Koga","year":"2009","unstructured":"Koga TMorita AMaruyama KLong-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation. Neuro Oncol. 2009;11(4):446-451."},{"issue":"7697","key":"bib6","series-title":"Nature","first-page":"469","article-title":"DNA methylation-based classification of central nervous system tumours","volume":"555","author":"Capper","year":"2018","unstructured":"Capper DJones DTWSill MDNA methylation-based classification of central nervous system tumours. Nature. 2018;555(7697):469-474."},{"issue":"3","key":"bib7","series-title":"JCO Precis Oncol","first-page":"1","article-title":"Successful targeting of an ATG7-RAF1 gene fusion in anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination","volume":"3","author":"Touat","year":"2019","unstructured":"Touat MYounan NEuskirchen PSuccessful targeting of an ATG7-RAF1 gene fusion in anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination. JCO Precis Oncol. 2019;3(3):1-7."},{"issue":"4","key":"bib8","series-title":"Histopathology","first-page":"375","article-title":"Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis","volume":"32","author":"Leonard","year":"1998","unstructured":"Leonard NAlcutt DAFarrell MA.Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis. Histopathology. 1998;32(4):375-378."},{"issue":"2","key":"bib9","series-title":"Childs Nerv Syst","first-page":"119","article-title":"Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation","volume":"20","author":"Lubansu","year":"2004","unstructured":"Lubansu ARorive SDavid PCerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. 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Expert Opin Drug Discov. 2016;11(9):907-916."},{"issue":"11","key":"bib24","series-title":"J Natl Compr Canc Netw","first-page":"1193","article-title":"BRAF\/MEK dual inhibitors therapy in progressive and anaplastic pleomorphic xanthoastrocytoma: case series and literature review","volume":"20","author":"Kata","year":"2022","unstructured":"Kata KRodriguez-Quintero JCArevalo ODBRAF\/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."},{"issue":"1","key":"bib25","series-title":"CNS Oncol","first-page":"5","article-title":"Dabrafenib in BRAFV600-mutated anaplastic pleomorphic xanthoastrocytoma","volume":"6","author":"Brown","year":"2017","unstructured":"Brown NFCarter TMulholland P.Dabrafenib in BRAFV600-mutated anaplastic pleomorphic xanthoastrocytoma. CNS Oncol. 2017;6(1):5-9."},{"issue":"6","key":"bib1","series-title":"Acta Neuropathol","first-page":"803","article-title":"The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary","volume":"131","author":"Louis","year":"2016","unstructured":"Louis DNPerry AReifenberger GThe 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131(6):803-820."},{"issue":"5","key":"bib2","series-title":"Brain Pathol","first-page":"575","article-title":"Pleomorphic xanthoastrocytoma: natural history and long-term follow-up","volume":"25","author":"Ida","year":"2015","unstructured":"Ida CMRodriguez FJBurger PCPleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol. 2015;25(5):575-586."},{"issue":"2","key":"bib3","series-title":"J Neurooncol","first-page":"343","article-title":"Impact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma","volume":"136","author":"Tonse","year":"2018","unstructured":"Tonse RGupta TEpari SImpact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma. J Neurooncol. 2018;136(2):343-350."},{"issue":"3","key":"bib4","series-title":"CNS Oncol","first-page":"CNS39","article-title":"Pleomorphic xanthoastrocytoma: a brief review","volume":"8","author":"Shaikh","year":"2019","unstructured":"Shaikh NBrahmbhatt NKruser TJPleomorphic xanthoastrocytoma: a brief review. CNS Oncol. 2019;8(3):CNS39."},{"issue":"4","key":"bib5","series-title":"Neuro Oncol","first-page":"446","article-title":"Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation","volume":"11","author":"Koga","year":"2009","unstructured":"Koga TMorita AMaruyama KLong-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation. Neuro Oncol. 2009;11(4):446-451."},{"issue":"7697","key":"bib6","series-title":"Nature","first-page":"469","article-title":"DNA methylation-based classification of central nervous system tumours","volume":"555","author":"Capper","year":"2018","unstructured":"Capper DJones DTWSill MDNA methylation-based classification of central nervous system tumours. Nature. 2018;555(7697):469-474."},{"issue":"3","key":"bib7","series-title":"JCO Precis Oncol","first-page":"1","article-title":"Successful targeting of an ATG7-RAF1 gene fusion in anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination","volume":"3","author":"Touat","year":"2019","unstructured":"Touat MYounan NEuskirchen PSuccessful targeting of an ATG7-RAF1 gene fusion in anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination. JCO Precis Oncol. 2019;3(3):1-7."},{"issue":"4","key":"bib8","series-title":"Histopathology","first-page":"375","article-title":"Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis","volume":"32","author":"Leonard","year":"1998","unstructured":"Leonard NAlcutt DAFarrell MA.Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis. Histopathology. 1998;32(4):375-378."},{"issue":"2","key":"bib9","series-title":"Childs Nerv Syst","first-page":"119","article-title":"Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation","volume":"20","author":"Lubansu","year":"2004","unstructured":"Lubansu ARorive SDavid PCerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. Childs Nerv Syst. 2004;20(2):119-122."},{"issue":"3","key":"bib10","series-title":"J Neurooncol","first-page":"431","article-title":"Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis\u2014a pediatric case report and review of the literature","volume":"94","author":"Okazaki","year":"2009","unstructured":"Okazaki TKageji TMatsuzaki KPrimary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis\u2014a pediatric case report and review of the literature. J Neurooncol. 2009;94(3):431-437."},{"issue":"9","key":"bib11","series-title":"J Clin Neurosci","first-page":"1299","article-title":"Spinal imaging in intracranial primary pleomorphic xanthoastrocytoma with anaplastic features","volume":"19","author":"Nern","year":"2012","unstructured":"Nern CHench JFischmann A.Spinal imaging in intracranial primary pleomorphic xanthoastrocytoma with anaplastic features. J Clin Neurosci. 2012;19(9):1299-1301."},{"issue":"8","key":"bib12","series-title":"J Clin Neurosci","first-page":"1370","article-title":"Anaplastic pleomorphic xanthoastrocytoma with spinal leptomeningeal spread at the time of diagnosis in an adult","volume":"22","author":"Benjamin","year":"2015","unstructured":"Benjamin CFaustin ASnuderl MPacione D.Anaplastic pleomorphic xanthoastrocytoma with spinal leptomeningeal spread at the time of diagnosis in an adult. 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