中耳神经源性肿瘤8例的诊治体会

Diagnosis and treatment of neurogenic tumors of middle ear: A report of 8 cases

摘要:

目的 探讨中耳神经源性肿瘤的临床特征、诊治方法及预后。方法 回顾性分析2010年1月—2023年3月收治的8例经病理确诊为中耳神经源性肿瘤患者的临床资料。8例患者首发症状中以耳流脓为主4例，听力下降3例，面瘫1例。5例肿物突入外耳道，3例鼓膜紧张部穿孔。纯音测听示8例患者均伴有不同程度的听力下降，其中混合性聋4例、传导性聋3例、极重度感音神经性聋1例。结果 所有患者均完善中耳CT及MRI，提示肿瘤累及中鼓室或鼓岬表面5例，累及面神经水平段3例，其中1例侵入中颅窝。8例患者均行乳突改良根治术，术后病理证实鼓室体副神经节瘤4例、面神经纤维瘤2例、面神经鞘膜瘤1例、中耳神经内分泌腺瘤1例；2例患者术前行活检分别诊断为中耳胆脂瘤和慢性化脓性中耳炎，术前病理误诊率为25%(2/8)。术后随访1个月至8年，目前尚无复发病例。结论 中耳神经源性肿瘤发病率较低且临床表现各异，存在一定的误诊率，术前完善CT和增强MRI检查以及术后病理及免疫组化有助于明确诊断。手术是治疗该病的有效手段。

Abstract:

Objective To investigate the clinical features, diagnosis and treatment methods as well as prognosis of neurogenic tumors in middle ear.Methods Clinical data of 8 patients with pathologically confirmed neurogenic tumors of the middle ear surgically treated in our hospital from Jan. 2010 to Mar. 2023 were retrospectively analyzed. Their initial symptoms were mainly ear discharge in 4 cases, hearing loss in 3 cases and facial paralysis in one. There were 5 cases with mass protrusion into the external auditory canal, 3 cases with tympanic tension perforation.Pure tone audiometry showed that all patients had varying degrees of hearing loss, including mixed deafness in 4 cases, conduction deafness in 3, and profound sensorineural deafness in one.Results Computerized tomography (CT) and magnetic resonance imaging (MRI) of the middle ear were completed in all patients, suggesting that the tumor involved the surface of promontory or the middle tympanum in 5 cases, and the horizontal segment of facial nerve in 3 cases with middle cranial fossa invasion in one. Modified radical mastoidectomy was performed in all the 8 patients, and the postoperative pathology confirmed tympanosoma in 4 cases, facial neurofibroma in 2 cases, facial neuroschwannoma in one and neuroendocrine adenoma in another. Two patients were diagnosed as middle ear cholesteatoma and chronic suppurative otitis media by preoperative biopsy with a preoperative pathological misdiagnosis rate of 25% (2/8). Postoperative followed up ranged from one month to 8 years revealed recurrence in none.Conclusions The incidence of neurogenic tumors in the middle ear is relatively low with different clinical manifestations and a certain misdiagnosis rate. Preoperative CT and enhanced MRI examination, as well as postoperative pathology and immunohistochemistry are helpful for definite diagnosis. Surgical treatment is an effective means to treat the disease.