内淋巴囊肿瘤的临床病理分析

Endolymphatic sac tumor: a clinicopathologic analysis

目的 探讨内淋巴囊肿瘤的临床病理学特征及诊断、鉴别诊断要点。方法 对 3例内淋巴囊肿瘤病例进行临床病理分析、PAS及免疫组织化学染色。结果 内淋巴囊肿瘤的临床症状包括进行性加重感音神经性聋、耳鸣、眩晕、颅神经受累等症状。镜下瘤组织呈乳头状囊性腺样结构 ,其内可见粉染蛋白样物质。部分瘤细胞的胞质透明 ,核的形态及大小相近 ,异型性较少见。肿瘤间质为大量增生的纤维结缔组织及透明变性的胶原纤维 ,可见出血、噬含铁血黄素细胞、异物巨细胞及胆固醇结晶。免疫表型 :CK、NSE阳性 ,GFAP、S 10 0蛋白、EMA、CgA、TG、Syn阴性。 结论 内淋巴囊肿瘤是罕见的肿瘤 。

Purpose To study the clinicopathologic features and differential diagnosis of the endolymphatic sac tumor. Methods Three cases of the endolymphatic sac tumor were studied with routine histopathological and immunohisochemical staining. Results The clinical symptoms of patients were progressive sensorineural deafness, tinnitus and vertigo. The histopathologic features presented papillary cystic pattern and some tumor cells with clear cytoplasm. Colloid material filled cysts, which was stained strongly with PAS. Hemorrhage, cholesterol crystal and foreign body giant cells were found in the stroma with abundant small blood vessels. The nuclei of tumor cells showed no apparent atypia. Immunohisochemically, the tumor cells were positive for CK and NSE, negative for GFAP, S 100, EMA, CgA, TG and Syn. Conclusions Endolymphatic sac tumor is a rare neoplasm. The diagnosis relies mainly on histopathological and immunohisochemical findings.