内淋巴囊肿瘤临床病理观察

Clinicopathological features of endolymphatic sac tumor

目的探讨内淋巴囊肿瘤(ELST)的病理形态特点、组织发生、诊断和鉴别诊断、治疗及预后,提高对该罕见肿瘤的认识。方法对1例ELST患者临床及病理资料进行分析,并行HE染色和免疫组化EnVision二步法。结果肿瘤大多数区域呈乳头状囊状结构,乳头状结构被覆无纤毛的单层立方-柱状上皮;部分区域呈腺样并呈囊性扩张,其内可见粉染的胶样物质,类似甲状腺滤泡内胶质。免疫组化示瘤细胞EMA、NSE、vimentin、VEGF、CK5/6和CK8/18(+)。结论 ELST较为罕见,临床及病理均易与其他肿瘤混淆,需要常规病理结合免疫组化进行诊断及鉴别诊断。

Objective To explore the morphological features,histogenesis,diagnosis and differential diagnosis,therapy and prognosis of the endolymphatic sac tumor（ELST） and to improve the understanding of this rare tumor.Methods The clinical and pathological data of one ELST was analyzed.The resected tumor was studied by using paraffin section,HE staining,and immunohistochemical staining（EnVision method）.Results Histologically,the tumor showed a papillary or cystic structure containing cuboidal or columnar cells with fibrous stroma and numerous microvessels.Some areas of the tumor showed adenoid or cystic structure,in which the pink staining substance mimic king thyroid colloid could be seen.By immunohistochemical stain,the tumor cells were mainly positive for EMA,NSE,vimentin,VEGF,CK5 /6 and CK8 / 18.Conclusion ELST is a rare tumor,which could be easily confused with other tumors in clinical or routinely pathologic work.The diagnosis and differential diagnosis rely mainly on histopathological and immunohistochemical findings.