内耳和桥小脑角罕见肿瘤及瘤样病变4例临床病理学分析  

Tumors and tumor-like lesions of internal auditory canal and cerebellopontine angle:a clinicopathologic study of 4 rare cases

作　　者：昌红[1] 陈奕至[1] 曲丛玲[1] 张建英[1] 沈兵[1] 高颖[1] 

机构地区：[1]北京世纪坛医院病理科,北京100038

出　　处：《临床与实验病理学杂志》2009年第2期162-165,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨以面瘫为主要表现的内耳和桥小脑角肿瘤和瘤样病变的病理形态特征。方法对4例以面瘫为主要表现的内耳和桥小脑角罕见肿瘤和瘤样病变进行光镜观察和免疫组化标记,并收集临床和影象资料并复习文献。结果1例为听神经脂肪瘤样迷离瘤(脂肪瘤样瘤),2例为内耳内淋巴囊来源的低级别乳头状腺癌,1例为类似肿瘤病变的Chievitz口旁器官。结论脂肪瘤样迷离瘤是一种发生在内耳听神经管或桥小脑角非常罕见的由脂肪、纤维、平滑肌、横纹肌以及神经成分组成的良性肿瘤。内耳内淋巴囊来源的低级别乳头状腺癌是发生在内耳内淋巴囊的特别罕见的低级别肿瘤。类似肿瘤病变的Chievitz口旁器官也非常罕见,是由上皮和间质成分构成的一种正常器官,常分布在颊神经的小分支周围,极易误诊为癌浸润外周神经和腺样囊性癌。Purpose To explore the clinicalpathological features of the tumor and tumor-like lesions of the internal auditary canal （IAC） and cerehellopontinc angle （CPA）. Methods The four cases of tumor of the IAC and CPA were studied by immunohistochemical staining and light microscopy, combined with MR1 and literature review. Results In four cases, one was lipochoristoma （lipomatous tumors） of the acoustic nerve, two were low-grade papillary adenoearcinoma of endolymphatic sac origin, and another one was juxtaoral organ of Chievitz presenting clinically as a tumor mass. Conclusions Lipomatous tumors in internal auditory canal and cerebellopontine angle are very rare; low-grade papillary adenocarcinoma of endolymphatic sac origin （Albeit locally aggressive） is rarely reported and has low malignant potential. Juxtaoral organ of Chievitz presents clinically as a tumor and may be mistaken as perineural invasion of carcinoma if the surgical pathologist is unaware of its existence.

关 键 词：桥小脑角肿瘤 内耳肿瘤 脂肪瘤样迷离瘤 乳头状腺癌 Chievitz口旁器官 

分 类 号：R739.61[医药卫生—肿瘤] R739.41[医药卫生—临床医学]