Primary melanoma of the cochlea with cerebellopontine extension and leptomeningeal spread
Primary melanoma of the cochlea with cerebellopontine extension and leptomeningeal spread
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DOI：
10.1017/s002221511400019x
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发表时间：
2014-07-01
影响因子：
1.7
通讯作者：
Dally, M.
中科院分区：
文献类型：
--
作者：
Koshy, A. N.;Briggs, R. J.;Dally, M.
背景资料：发生在内耳道和桥小脑角区域的肿瘤中，恶性肿瘤不到1%。原发性中枢神经系统黑色素瘤从这一地区是非常罕见的，往往最初被误诊为听神经mangeros.Methods：我们提出了一个71岁的男子急性前庭障碍和单侧听力损失。磁共振成像显示一个肿块，被认为是耳蜗神经鞘瘤，涉及耳蜗和内听道。在手术中，一个色素肿块粘附面神经可视化，观察到的组织病理学是一致的恶性黑色素瘤。没有颅外部位的原发性肿瘤被发现，提示原发性中枢神经系统melanoma.Results：尽管手术切除和辅助放疗，病人重新提出了广泛的软脑膜diseases16个月later.Conclusion：恶性肿瘤在内耳道和桥小脑角区是罕见的。早期诊断和管理是通过识别特征性因素，如既往恶性肿瘤史，非典型磁共振成像结果和加速听前庭症状。尽管患者的结果，全手术切除术与术后放疗仍然是推荐的治疗。
Background: Less than 1 per cent of tumours occurring in the region encompassing the internal auditory canal and the cerebellopontine angle are malignant. Primary central nervous system melanomas arising from this region are exceptionally rare and are often initially misdiagnosed as acoustic neuromas.Methods: We present a 71-year-old man with acute vestibular disturbance and unilateral hearing loss. Magnetic resonance imaging demonstrated a mass, thought to be a cochlear nerve schwannoma, involving the cochlea and the internal auditory canal. At surgery, a pigmented mass adherent to the facial nerve was visualised, and the observed histopathology was consistent with a malignant melanoma. No extracranial site for the primary tumour was found, suggestive of a primary central nervous system melanoma.Results: Despite surgical resection and adjuvant radiotherapy, the patient re-presented with extensive leptomeningeal disease 16 months later.Conclusion: Malignant tumours in the internal auditory canal and cerebellopontine angle region are rare. Early diagnosis and management are aided by recognition of characteristic factors such as a history of prior malignancy, atypical magnetic resonance imaging findings and accelerated audiovestibular symptoms. Despite the presented patient's outcome, total surgical resection with post-operative radiotherapy remains the recommended treatment.