Bilateral cerebellopontine angle lesions not always NF2: diagnostic pitfall

Bilateral cerebellopontine angle lesions not always NF2: diagnostic pitfall
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双侧桥小脑角病变并不总是 NF2:诊断陷阱

DOI:
10.3109/02688697.2011.603851
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发表时间:
2012
影响因子:
1.1
通讯作者:
M. Kamel
M. Kamel
中科院分区:
医学4区
文献类型:
--
作者:
A. Rao;A. Lawrie;P. Bodkin;J. Tighe;M. Kamel

文献摘要

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双侧内耳道(IAC)肿瘤几乎完全与双侧前庭神经鞘瘤相关,很少有,如果有的话,可以模仿这种外观。我们报告一个非常罕见的病例,一位75岁的男士,他最初表现为双侧IAC肿瘤,后来被诊断为孤立的原发性中枢神经系统骨髓瘤,没有全身受累。这是一种非常罕见的表现,具有重要的诊断和治疗意义。他接受来那度胺和地塞米松联合治疗。治疗耐受性良好,但反应有限。虽然罕见,但转移应被视为IAC病变的鉴别诊断。
Abstract Bilateral internal auditory canal (IAC) tumours are almost exclusively associated with bilateral vestibular schwannomas, and there is very little, if anything, that can mimic this appearance. We present a very rare case of a 75-year-old gentleman who initially presented with bilateral IAC tumours and later diagnosed as an isolated primary CNS myeloma without systemic involvement. This is a very rare presentation and has important diagnostic and therapeutic implications. He was treated with a combination of lenalidomide and dexamethasone. The treatment was well tolerated but with limited response. Although rare, metastasis should be considered as a differential diagnosis of IAC lesions.