Margossian Khatcher O, Marciniak Marcin, Al-Warqi Akram, Aldape Kenneth, Kocak Mehmet, Munoz Lorenzo, Michaelides Elias, Marshall Michael S
Department of Pathology, Rush University Medical Center, Chicago, Illinois.
Department of Otolaryngology-Head and Neck Surgery, Rush University Medical Center, Chicago, Illinois.
J Neurosurg Case Lessons. 2025 May 26;9(21). doi: 10.3171/CASE2586.
Myxopapillary ependymomas (MPEs) are a relatively rare subtype of ependymoma that occur almost exclusively at the conus medullaris and filum terminale in the lumbosacral spine. Intracranial occurrences of this tumor are rare, and of those, localization to the cerebellopontine angle (CPA) is an exceptional event. To the authors' knowledge, this is the fifthreported MPE in the CPA and only the third reported instance of an MPE in the CPA at the initial presentation.
The authors describe an adult patient with an initial presentation of MPE at the CPA, originally thought to be a schwannoma on radiological imaging. Follow-up imaging later revealed additional clinically asymptomatic spinal masses consistent with MPEs. This case is particularly unique not only because of the patient's presenting symptoms being exclusive to tinnitus and ear fullness without any spinal symptoms, but also because it is the first case, to the authors' knowledge, in which DNA methylation profiling results have been reported to confirm the diagnosis of MPE in the CPA.
This case highlights the importance of considering a broad differential diagnosis for lesions in the CPA. https://thejns.org/doi/10.3171/CASE2586.
黏液乳头型室管膜瘤（MPE）是室管膜瘤中一种相对罕见的亚型，几乎仅发生于腰骶部脊柱的脊髓圆锥和终丝。该肿瘤发生于颅内者罕见，其中，位于桥小脑角（CPA）者更是罕见。据作者所知，这是第5例报道的CPA区MPE，且是仅有的第3例在初次就诊时即报道的CPA区MPE。
作者描述了1例成年患者，最初表现为CPA区的MPE，影像学检查最初认为是神经鞘瘤。后续影像学检查后来发现了其他与MPE一致的临床无症状性脊柱肿块。该病例特别独特，不仅因为患者的症状仅为耳鸣和耳闷胀感而无任何脊柱症状，还因为据作者所知，这是首例报道了DNA甲基化谱分析结果以证实CPA区MPE诊断的病例。
该病例强调了对CPA区病变进行广泛鉴别诊断的重要性。https://thejns.org/doi/10.3171/CASE2586
