{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T00:05:42Z","timestamp":1789517142202,"version":"build-2803163510"},"reference-count":17,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"11","license":[{"start":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T00:00:00Z","timestamp":1789344000000},"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 Neurosurg Case Lessons"],"published-print":{"date-parts":[[2026,9,14]]},"abstract":"\n BACKGROUND<\/jats:title>\n \n FET-CREB<\/jats:italic>\n (\n EWSR1-ATF1<\/jats:italic>\n ) fusion\u2013positive intracranial mesenchymal tumors (IMTs) are locally aggressive with a high recurrence rate, particularly for pediatric patients. Management is challenging because of their complex diagnostic criteria, tumor location, and limited evidence of effective adjuvant therapy. The authors present the case of a child with multiple recurrences of\n FET-CREB<\/jats:italic>\n fusion\u2013positive IMT.\n <\/jats:p>\n <\/jats:sec>\n \n OBSERVATIONS<\/jats:title>\n \n A 6-year-old male with cerebral palsy presented with 3 weeks of vomiting, gait decline, and headaches. Physical examination revealed right-sided weakness, facial asymmetry, and dysphagia. Imaging revealed a large right-sided cystic cerebellar mass extending to the cerebellopontine angle, with mass effect on the brainstem and obstructive hydrocephalus. He underwent multiple resections for recurrent disease via a right retrosigmoid craniotomy\/craniectomy. Histopathological and molecular testing confirmed an\n FET-CREB<\/jats:italic>\n fusion\u2013positive IMT. Efforts toward gross-total resection were limited by the tumor abutting cranial nerves (CNs) VII and VIII. Small residual tumor was intentionally left to avoid neurological decline. Given subtotal resection, adjuvant therapy was initiated. The patient required multidisciplinary rehabilitation and ongoing management for right-sided facial droop and CN VII palsy.\n <\/jats:p>\n <\/jats:sec>\n \n LESSONS<\/jats:title>\n \n This case highlights local aggression of\n FET-CREB<\/jats:italic>\n fusion\u2013positive IMTs and the resection preservation trade-off. Molecular confirmation, multimodal care, and novel effective adjuvant therapy are essential.\n <\/jats:p>\n \n https:\/\/thejns.org\/doi\/10.3171\/CASE26367<\/jats:ext-link>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/case26367","type":"journal-article","created":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T00:00:30Z","timestamp":1789516830000},"source":"Crossref","is-referenced-by-count":0,"title":["Mesenchymal tumors of the central nervous system: illustrative case"],"prefix":"10.3171","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-8713-5658","authenticated-orcid":true,"given":"Michael K.","family":"Anyane-Yeboa","sequence":"first","affiliation":[{"name":"Albert Einstein College of Medicine, Bronx;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiashu","family":"Wang","sequence":"additional","affiliation":[{"name":"Albert Einstein College of Medicine, Bronx;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaitlyn","family":"Quijano","sequence":"additional","affiliation":[{"name":"Albert Einstein College of Medicine, Bronx;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger","family":"Fecher","sequence":"additional","affiliation":[{"name":"Department of Pathology, Montefiore Medical Center, Bronx"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Kobets","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Lenox Hill Hospital, Manhattan, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"bib1","series-title":"Front Oncol","first-page":"1203994","article-title":"Case report: remarkable breakthrough: successful treatment of a rare intracranial mesenchymal, FET::CREB fusion-positive tumor treated with patient-\u00adtailored multimodal therapy","volume":"13","author":"D\u2019Antonio","year":"2023","unstructured":"D\u2019Antonio FRossi SGiovannoni ICase report: remarkable breakthrough: successful treatment of a rare intracranial mesenchymal, FET::CREB fusion-positive tumor treated with patient-\u00adtailored multimodal therapy. Front Oncol. 2023;13:1203994. doi:10.3389\/fonc.2023.1203994"},{"issue":"4","key":"bib2","series-title":"Am J Surg Pathol","first-page":"482","article-title":"EWSR1 fusions with CREB family transcription factors define a novel myxoid mesenchymal tumor with predilection for intracranial location","volume":"41","author":"Kao","year":"2017","unstructured":"Kao YCSung YSZhang LEWSR1 fusions with CREB family transcription factors define a novel myxoid mesenchymal tumor with predilection for intracranial location. Am J Surg Pathol. 2017;41(4):482-490. doi:10.1097\/PAS.0000000000000788"},{"issue":"6","key":"bib3","series-title":"Pathologica","first-page":"455","article-title":"Expanding the spectrum of \u201cmesenchymal\u201d tumors of the central nervous system","volume":"114","author":"Pizzimenti","year":"2022","unstructured":"Pizzimenti CGianno FGessi M.Expanding the spectrum of \u201cmesenchymal\u201d tumors of the central nervous system. Pathologica. 2022;114(6):455-464. doi:10.32074\/1591-951X-826"},{"issue":"2","key":"bib4","series-title":"Acta Neurol Belg","first-page":"691","article-title":"Angiomatoid fibrous histiocytoma: an atypical brain location newly described as intracranial mesenchymal tumor FET-CREB fusion-positive","volume":"123","author":"Lemnos","year":"2023","unstructured":"Lemnos LSalle HCaire FDuchesne M.Angiomatoid fibrous histiocytoma: an atypical brain location newly described as intracranial mesenchymal tumor FET-CREB fusion-positive. Acta Neurol Belg. 2023;123(2):691-694. doi:10.1007\/s13760-022-01966-5"},{"key":"bib5","series-title":"World Neurosurg X","first-page":"100349","article-title":"Central nervous system tumors of uncertain differentiation","volume":"22","author":"Jacobo","year":"2024","unstructured":"Jacobo JA.Central nervous system tumors of uncertain differentiation. World Neurosurg X. 2024;22:100349. doi:10.1016\/j.wnsx.2024.100349"},{"issue":"8","key":"bib6","series-title":"Nat Rev Clin Oncol","first-page":"509","article-title":"Challenges to curing primary brain tumours","volume":"16","author":"Aldape","year":"2019","unstructured":"Aldape KBrindle KMChesler LChallenges to curing primary brain tumours. Nat Rev Clin Oncol. 2019;16(8):509-520. doi:10.1038\/s41571-019-0177-5"},{"issue":"16","key":"bib7","series-title":"J Clin Oncol","first-page":"2306","article-title":"Increased permeability of the blood-brain barrier to chemotherapy in metastatic brain tumors: establishing a treatment paradigm","volume":"25","author":"Gerstner","year":"2007","unstructured":"Gerstner ERFine RL.Increased permeability of the blood-brain barrier to chemotherapy in metastatic brain tumors: establishing a treatment paradigm. J Clin Oncol. 2007;25(16):2306-2312. doi:10.1200\/JCO.2006.10.0677"},{"issue":"1","key":"bib8","series-title":"Acta Neuropathol Commun","first-page":"17","article-title":"Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor","volume":"12","author":"Mezzacappa","year":"2024","unstructured":"Mezzacappa FMSmith FKZhang WPotential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor. Acta Neuropathol Commun. 2024;12(1):17. doi:10.1186\/s40478-024-01721-2"},{"issue":"1","key":"bib9","series-title":"Brain Pathol","first-page":"e13010","article-title":"An integrative histopathological and epigenetic characterization of primary intracranial mesenchymal tumors, FET:CREB-fused broadening the spectrum of tumor entities in comparison with their soft tissue counterparts","volume":"32","author":"Tauzi\u00e8de-Espariat","year":"2022","unstructured":"Tauzi\u00e8de-Espariat ASievers PLarousserie FAn integrative histopathological and epigenetic characterization of primary intracranial mesenchymal tumors, FET:CREB-fused broadening the spectrum of tumor entities in comparison with their soft tissue counterparts. Brain Pathol. 2022;32(1):e13010. doi:10.1111\/bpa.13010"},{"issue":"5","key":"bib10","series-title":"EMBO Rep","first-page":"e45766","article-title":"FET family fusion oncoproteins target the SWI\/SNF chromatin remodeling complex","volume":"20","author":"Lind\u00e9n","year":"2019","unstructured":"Lind\u00e9n MThomsen CGrundevik PFET family fusion oncoproteins target the SWI\/SNF chromatin remodeling complex. EMBO Rep. 2019;20(5):e45766. doi:10.15252\/embr.201845766"},{"issue":"4","key":"bib11","series-title":"Brain Pathol","first-page":"e12918","article-title":"Intracranial mesenchymal tumor with FET-CREB fusion\u2014a unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms","volume":"31","author":"Sloan","year":"2021","unstructured":"Sloan EAChiang JVillanueva-Meyer JEIntracranial mesenchymal tumor with FET-CREB fusion\u2014a unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms. Brain Pathol. 2021;31(4):e12918. doi:10.1111\/bpa.12918"},{"issue":"7","key":"bib12","series-title":"Am J Surg Pathol","first-page":"e1","article-title":"Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status","volume":"36","author":"Thway","year":"2012","unstructured":"Thway KFisher C.Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status. Am J Surg Pathol. 2012;36(7):e1-e11. doi:10.1097\/PAS.0b013e31825485c5"},{"issue":"suppl 4","key":"bib13","series-title":"Neuro Oncol","first-page":"0","article-title":"OTHR-08. Clinical management of two pediatric cases of intracranial mesenchymal tumors with FET","volume":"26","author":"Lucas","year":"2024","unstructured":"Lucas BJMcGovern SLWhitehead WEOTHR-08. Clinical management of two pediatric cases of intracranial mesenchymal tumors with FET. Neuro Oncol. 2024;26(suppl 4):0. doi:10.1093\/neuonc\/noae064.679"},{"issue":"1","key":"bib14","series-title":"Neurooncol Adv","first-page":"vdad026","article-title":"Intracranial mesenchymal tumor, FET::CREB fusion-positive in the lateral ventricle","volume":"5","author":"Ozkizilkaya","year":"2023","unstructured":"Ozkizilkaya HIJohnson JMO\u2019Brien BJIntracranial mesenchymal tumor, FET::CREB fusion-positive in the lateral ventricle. Neurooncol Adv. 2023;5(1):vdad026. doi:10.1093\/noajnl\/vdad026"},{"issue":"5","key":"bib15","series-title":"Pediatr Neurosurg","first-page":"347","article-title":"Intracranial myxoid mesenchymal tumor with rare EWSR1-CREM translocation","volume":"54","author":"White","year":"2019","unstructured":"White MDMcDowell MMPearce TMBukowinski AJGreene S.Intracranial myxoid mesenchymal tumor with rare EWSR1-CREM translocation. Pediatr Neurosurg. 2019;54(5):347-353. doi:10.1159\/000501695"},{"issue":"12","key":"bib16","series-title":"Pathol Res Pract","first-page":"153239","article-title":"Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion","volume":"216","author":"Libbrecht","year":"2020","unstructured":"Libbrecht SVan Der Meulen JMondelaers VIntracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion. Pathol Res Pract. 2020;216(12):153239. doi:10.1016\/j.prp.2020.153239"},{"issue":"3","key":"bib17","series-title":"Neuropathology","first-page":"244","article-title":"Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL-6\/STAT3 signaling","volume":"43","author":"Hojo","year":"2023","unstructured":"Hojo KFuruta TKomaki SSystemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL-6\/STAT3 signaling. Neuropathology. 2023;43(3):244-251. doi:10.1111\/neup.12877"}],"container-title":["Journal of Neurosurgery: Case Lessons"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-case-lessons\/12\/11\/article-CASE26367.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-case-lessons\/12\/11\/article-CASE26367.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-case-lessons\/12\/11\/article-CASE26367.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T00:00:30Z","timestamp":1789516830000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-case-lessons\/12\/11\/article-CASE26367.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,14]]},"references-count":17,"journal-issue":{"issue":"11"},"URL":"https:\/\/doi.org\/10.3171\/case26367","relation":{},"ISSN":["2694-1902"],"issn-type":[{"value":"2694-1902","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,14]]}}}