内淋巴囊肿瘤临床病理及影像特点分析

Clinicopathological and imaging feature of endolymphatic sac tumor

目的探讨内淋巴囊肿瘤的临床病理、影像特征、诊断要点及肿瘤的预后情况。方法对7例内淋巴囊肿瘤病例进行临床、影像及病理分析,并对患者进行随访,观察肿瘤预后及患者生存情况。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耳聋、耳鸣、眩晕、脑神经受累等。CT检查可见以岩骨后缘中部为中心的蜂窝状或虫蚀状骨质破坏。MRI检查可见不增强的T1WI及T2MI上所有肿瘤的边缘显示高信号密度,肿瘤实质显示低信号密度,并可见血管流空信号。镜下瘤组织主要呈乳头状结构,局部可见腺样及囊性结构,部分腺腔及囊腔内可见粉染胶样物质,肿瘤细胞为圆形或立方形,部分瘤细胞的胞质透明,核的形态及大小比较一致,异型性不明显。肿瘤间质血管较丰富,且胶原及纤维结缔组织增生,可见出血、噬含铁血黄素细胞、异物巨细胞及胆固醇结晶,部分病例出现骨质破坏及坏死。免疫表型:CK、NSE阳性,部分病例CD56、S-100蛋白阳性,GFAP、TTF-1、CgA、TG、Syn均阴性。7例患者中,1例肿瘤局部切除后行放射治疗,另1例第1次手术后两年复发,其余患者均未见复发、转移。结论内淋巴囊肿瘤较罕见,主要依靠病理组织学和免疫组化法诊断和鉴别诊断,患者的预后情况较好。

Objective To study the clinicopathological and imaging features,key points of diagnosis and prognosis of endolymphatic sac tumor.Methods Seven cases of endolymphatic sac tumor were studied with routine histopathological,immunohisochemical staining and CT,MRI scanning.Patients＇ prognosis and life quality were aesessed by follow-up.Results The clinical symptoms of patients were progressive sensorineural deafness,tinnitus,vertigo and affection of cranial nerves.CT revealed reticular of moth-eaten bony destruction of the petrosal region,and MRI revealed a region of high-signal intensity void of vascular flowing signals.The histopathologic examination of the tumor presented a papillary-cystic pattern.The tumor cells were round or cubaid in shapen,with nuclei in similar shape and size.There were cyst-like structares filled mith pinkish gelatinous.Tumor cells were roundor cuboid in shape,and some of them contained clear cytofrlasm.There was materials,which were stained strongly with PAS.Hemorrhage,cholesterol crystal and foreign body giant cells were found in the tumor stroma with abundant small blood vessels and extravasation of blood,cells with intracellular hematin,and giant cells collagen.There was also proliferation of fibrous connective tissues.Necrosis and bone destruction were also found in some cases.Immunohistochemical results showed that CK and NSE were positive in all the tumors,and CD56 and S-100 were positive in part of cases,GFAP,EMA,CgA,TG and Syn were negative in all the specimens.One patient had received radiotherapy after partial resection.Relapse occurred only in one patient two years after the first surgery,and no metastasis or relapse occurred in the rest of patients.Conclusions Endolymphatic sac tumor is a rare neoplasm.The diagnosis relies mainly on histopathological and immunohisochemical findings.The patients often have good prognosis.