Lee Jong Im, Kim Ki Kwon, Park Yoon Keun, Eah Kyung Yoon, Kim Jung Ran
Department of Pathology, College of Medicine, Dongguk University, 707 Sukjang-dong, Gyeongju 780-714, Korea.
J Korean Med Sci. 2004 Feb;19(1):155-8. doi: 10.3346/jkms.2004.19.1.155.
Heterotopic brain tissue usually involves extracranial midline structures of the head and neck such as nose, nasopharynx, and oral cavity. Its occurrence in the non-midline structures, including middle ear, is rare. We described a 50-yr-old-man with heterotopic glial tissue in the middle ear and mastoid bone. The patient presented with progressive hearing loss for 8 yr. There was no history of congenital anomalies, trauma, or ear surgery. Computed tomography revealed a mass-like lesion with soft tissue density occupying the middle ear cavity and mastoid antrum. At the operation, a gray-white fibrotic mass was detected in the epitympanic area. Mesotympanum and ossicles were intact. The patient underwent left simple mastoidectomy with type I tympanoplasty. During operation, definite cranial bone defect or cerebrospinal fluid leakage was not found. Histologically, the lesion was composed of exclusively mature, disorganized glial tissue with fibrovascular elements in a rather loose fibrillary background. Glial tissue showed diffuse positive reaction for glial fibrillar acidic protein and S100 protein on immunohistochemical study.
异位脑组织通常累及头颈部的颅外中线结构,如鼻子、鼻咽部和口腔。其在包括中耳在内的非中线结构中发生较为罕见。我们描述了一名50岁男性,中耳和乳突骨存在异位胶质组织。患者有8年渐进性听力损失。无先天性异常、外伤或耳部手术史。计算机断层扫描显示一个软组织密度的肿块样病变占据中耳腔和乳突窦。手术时,在上鼓室区域发现一个灰白色纤维化肿块。中鼓室和听小骨完好。患者接受了左侧单纯乳突切除术及I型鼓室成形术。手术过程中未发现明确的颅骨缺损或脑脊液漏。组织学上,病变仅由成熟、杂乱的胶质组织组成,在相当疏松的纤维背景中有纤维血管成分。免疫组织化学研究显示,胶质组织对胶质纤维酸性蛋白和S100蛋白呈弥漫性阳性反应。