{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T11:25:01Z","timestamp":1768476301665,"version":"3.49.0"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T00:00:00Z","timestamp":1698883200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP23K08559"],"award-info":[{"award-number":["JP23K08559"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:p>Embryonal tumors with multilayered rosettes (ETMRs) are aggressive central nervous system (CNS) tumors that usually occur in young children. Here, we describe the first incidence of ETMR in an adult patient that also originated in the novel location of the internal auditory canal (IAC). The 36\u2010year\u2010old patient initially presented with unsteadiness, diplopia, and tinnitus. The tumor in the IAC was discovered on brain magnetic resonance imaging, and gross total resection was performed followed by pathological and molecular diagnosis. The patient received whole brain and spinal cord radiotherapy after an intracranial recurrence and adjuvant chemotherapy consisting of four cycles of ifosfamide, cisplatin, and etoposide. Progression was rapid; however, the patient survived for 22\u2009months after diagnosis before succumbing to the disease. Molecular investigation revealed a <jats:italic>DICER1<\/jats:italic> mutation at exon 25, and methylation classification categorized the tumor as ETMR, non\u2010C19MC\u2010altered. This case underscores the diverse possible presentations of ETMR, <jats:italic>DICER1\u2010<\/jats:italic>mutated and the importance of molecular techniques to characterize and promptly treat atypical ETMR.<\/jats:p>","DOI":"10.1111\/neup.12951","type":"journal-article","created":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T06:05:34Z","timestamp":1698991534000},"page":"208-215","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Embryonal tumor with multilayered rosettes arising from the internal auditory canal of an adult: Illustrative case with molecular investigations"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7681-7744","authenticated-orcid":false,"given":"Adam","family":"Sheriff","sequence":"first","affiliation":[{"name":"Guy's, King's and St Thomas' (GKT), King's College London  London 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