Department of Neurosurgery, Sichuan Provincial People's Hospital, Chengdu, Sichuan Province, China.
J Neurosurg. 2011 May;114(5):1288-93. doi: 10.3171/2010.12.JNS10912. Epub 2011 Jan 21.
Gliomas are rare entities in the cerebellopontine angle (CPA) in adults. The authors present clinical, neuroradiological, serological, and neuropathological findings in a 60-year-old man with an extraaxial CPA glioblastoma arising from the proximal portion of cranial nerve VIII. The patient presented with progressive left-sided deafness and left-sided facial palsy lasting less than 2 months and progressive dysarthria and dysphagia lasting 2 weeks. Preoperative neuroimaging suggested the diagnosis of CPA meningioma with "dural-tail" sign and involvement of the internal auditory canal. Serological examination showed an increase in the malignant markers of ferritin and neuron-specific enolase, which suggested underlying malignancy. The tumor was subtotally removed, and it was confirmed to be completely separated from the brainstem and cerebellum. Cranial nerves VII and VIII were destroyed and sacrificed. Transient severe bradycardia occurred during surgery due to entrapment of the caudal cranial nerve complex by the tumor in such an infiltrative way. The neuropathological examination revealed a glioblastoma. The patient underwent no further treatment and died of cachexia 2 months postoperatively. To the authors' knowledge, this represents the first case of a primary glioblastoma in the CPA in an adult. A high index of suspicion along with reliance on clinical assessment, radiological findings, and serum detection of specific malignant markers is essential to diagnose such uncommon CPA lesions.
成人桥小脑角(CPA)的神经胶质瘤很少见。作者报告了一位 60 岁男性的临床、神经影像学、血清学和神经病理学发现,该患者的颅神经 VIII 近端有外轴性 CPA 胶质母细胞瘤。该患者表现为进行性左侧耳聋和左侧面瘫持续时间不到 2 个月,以及进行性构音障碍和吞咽困难持续 2 周。术前神经影像学提示 CPA 脑膜瘤的诊断,具有“硬膜尾”征和内听道受累。血清学检查显示铁蛋白和神经元特异性烯醇化酶等恶性标志物增加,提示潜在恶性。肿瘤被部分切除,并证实与脑干和小脑完全分离。颅神经 VII 和 VIII 被破坏并牺牲。由于肿瘤呈浸润性生长,尾端颅神经复合体被肿瘤包裹,术中出现短暂性严重心动过缓。病理检查显示为胶质母细胞瘤。患者未接受进一步治疗,术后 2 个月死于恶病质。据作者所知,这是首例成人 CPA 原发性胶质母细胞瘤。诊断这种罕见的 CPA 病变时,需要高度怀疑并依赖临床评估、影像学发现和血清特定恶性标志物的检测。