摘要：
目的 观察中耳腺瘤的临床病理学特点,增强对中耳腺瘤的认识.方法 收集首都医科大学附属北京同仁医院2004年至2014年8例中耳腺瘤的临床病理资料,行免疫组织化学EnVision法染色,并对患者进行随访.结果 8例患者女性5例,男性3例.发病年龄21 ～51岁,平均年龄37.5岁,中位年龄37岁.患者均为单侧发病,左侧5例,右侧3例.7例首次发病患者的临床表现包括听力下降(6例)、耳鸣(5例)、耳闷感(3例)、耳痛(1例)、面瘫(1例),术前诊断包括慢性中耳炎和/或中耳占位(7例)、周围性面瘫(1例).1例因术后复发就诊患者临床表现为耳流水伴耳闷感、耳鸣.组织学上,肿瘤细胞呈实性片状、巢状、腺样、缎带样及小梁状等多种方式排列,仅1例以腺样结构为主,另外7例均以非腺样结构为主.免疫组织化学染色,肿瘤细胞弥漫表达CK(8/8)、波形蛋白(8/8),局部表达CK7 (8/8)、CK5/6(8/8).CK7、CK5/6主要在腺样结构区表达,CK7表达于腺样结构的腔面,CK5/6表达于腺样结构的基底面,二者有时可在局部非腺样结构区呈散在阳性.突触素呈弥漫阳性(8/8),神经元特异性烯醇化酶呈弥漫弱阳性(5/8),嗜铬粒素A呈弥漫或局灶散在阳性(4/8).S-100蛋白、Calponin均阴性.Ki-67阳性指数低,约1％～2％.随访6例,随访时间1年3个月至10年6个月,平均随访时间4年2个月,复发2例,无局部转移及远处转移.结论 中耳腺瘤临床表现不特异,确诊需依靠病理检查.其组织形态多样,镜下应注意与中耳其他占位性病变鉴别,免疫组织化学染色有助于诊断及鉴别诊断.中耳腺瘤预后良好,少数病例有复发可能,术后需进行长期随访.%Objective To investigate the clinical and pathologic features of middle ear adenoma (MEA).Methods Eight cases of MEA were collected from Beijing Tongren Hospital,Capital Medical University between 2004 and 2014,and immunohistochemical staining was performed.Results The patients included five women and three men (mean age,37.5 years; median 37 years; range,21-51 years).All patients had unilateral lesions.Five MEA occurred on the left side,and three on the right.In seven patients the MEA was primary,and they presented with hearing loss (6 cases),tinnitus (5 cases),sense of ear blockage (3 cases),otalgia (1 case) and facial nerve paralysis (1 case).The remaining patient had recurrent MEA,and presented with otorrhea,aural fullness and tinnitus.Histologically,the tumor cells were arranged in a variety of patterns,including solid sheets,nests,glands,ribbons or trabeculae.Glandular structures were prominent in one case only.Immunohistochemically,the tumor cells were diffusely positive for keratin (8/8) and vimentin (8/8),and focally positive for CK 7 (8/8) and CK5/6(8/8).CK7 and CK5/6 were predominantly positive in tumor cells with glandular growth pattern; CK7 was positive in the luminal cells while CK5/6 was positive in the abluminal cells.Both were also expressed focally in scattered tumor cells with non-glandular pattern.The tumor cells were also diffusely positive for synaptophysin (8/8),diffusely but weakly positive for NSE (5/8),and were diffusely or focally positive for chromogranin A (4/8).Both S-100 protein and calponin were negative in all cases.The proliferation rate was low,about 1％-2％.Six cases were followed up for one year and three months to ten years and six months,with an average follow-up period of four years and two months.Two patients developed recurrence,but there were no regional or distant metastases.Conclusions Diagnosis of MEA requires pathologic confirmation since the clinical symptoms are non-specific.MEA can show a variety of histologic patterns,and should be distinguished from other space-occupying lesions at this site.Immunohistochemical staining has greatly contributed to the diagnosis and differential diagnosis of MEA.The prognosis of this tumor is good.Patients with MEA require long-term follow-up for recurrences.
