Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Center of Neurosensorial-Head and Neck Diseases, Lariboisière Hospital, University Paris 7 Paris Diderot, Assistance Publique des Hôpitaux de Paris & EA7334 Patient-Reported Outcomes, University Paris 7 Diderot University, Sorbonne Paris Cité, Paris.
Department of Neuroradiology, Rouen University Hospital, Rouen, France.
Otol Neurotol. 2019 Oct;40(9):1237-1245. doi: 10.1097/MAO.0000000000002360.
Metastatic lesions to the internal auditory meatus (IAM) and/or the cerebellopontine angle (CPA) are rare and may appear like a vestibular schwannoma (VS). We herein raise the issue of the diagnosis and treatment of nine malignant cases of the CPA and IAM among three referral centers in France and Japan. The aim of this study was 1) to report malignant lesions of the CPA, their diagnosis and treatment, 2) to review the literature, 3) to propose criteria of suspicion for malignant tumors of the CPA.
Nine patients who had malignant lesions of the CPA and/or IAM for whom the final diagnosis was made by surgery, lumbar puncture, or PET scan were included. The main outcomes measured were: rapid onset of symptoms, association of cochlea-vestibular symptoms with facial palsy, and MRI analysis.
Among the nine patients with malignant tumor of the CPA, 8 of them (89%) had a facial palsy associated with cochlea-vestibular symptoms. Rapid growth of the tumor was observed in 77% (7/9) of the cases in a mean time interval of 4.6 months. The initial diagnosis evoked was VS in 44% of the cases (4/9). Atypical MRI aspect was seen in 67% of the cases (6/9) with bilateral tumors in 55% of cases (5/9).
Although rare, malignant tumors of the CPA and/or IAM should be evoked in case of association of cochleovestibular symptoms and facial palsy, rapid onset and atypical MRI aspect.
内听道(IAM)和/或桥小脑角(CPA)的转移病变很少见,可能表现为前庭神经鞘瘤(VS)。我们在此提出了法国和日本的三个转诊中心的 9 例恶性 CPA 和 IAM 病例的诊断和治疗问题。本研究的目的是:1)报告 CPA 的恶性病变及其诊断和治疗,2)复习文献,3)提出 CPA 恶性肿瘤可疑的标准。
纳入了 9 例最终通过手术、腰椎穿刺或 PET 扫描确诊为 CPA 和/或 IAM 恶性病变的患者。主要观察指标为:症状迅速出现、耳蜗-前庭症状与面瘫的相关性以及 MRI 分析。
在 9 例 CPA 恶性肿瘤患者中,8 例(89%)有耳蜗-前庭症状伴面瘫。77%(7/9)的病例肿瘤在平均 4.6 个月的时间内迅速生长。最初诊断为 VS 的病例占 44%(4/9)。67%(6/9)的病例 MRI 表现不典型,55%(5/9)的病例为双侧肿瘤。
尽管罕见,但当出现耳蜗-前庭症状和面瘫、快速发病和不典型 MRI 表现时,应考虑 CPA 和/或 IAM 的恶性肿瘤。