Clinicopathological and imaging feature of endolymphatic sac tumor / 解放军医学杂志

ABSTRACT

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.