Clinicopathological features of low-grade malignant endolymphatic sac adenocaricinomas

WANG Hong-qun; LI Jie; SHI Huai-yin

doi:10.3969/j.issn.1007-8096.2018.02.004

Abstract：Objective To explore the clinical-pathological characteristics of endolymphatic sac tumors (ELSTs) Methods The clinicopathological data were collected and analyzed in 21 cases of ELSTs.Results The patients were aged 16-71 years (median:39 years;mean:40.3 years).There were 13 famales and 8 males,yielding a female/male ratio of 1.6∶1.1 cases were located left side which were predominante,7 for right side,1 for bilateral one.Type O and B blood of the female patients occupied 71.4％.Immunohistochemically,CK,EMA and vimentin were all positive.S-100 (10/14),CD56(9/12),NSE(2/4),GFAP(1/11) were partially positive;Syn,CgA,TTF-1,TG,CD34 and calcitonin were negative.The average Ki-67 indexes were 4.3％.The histologic figures showed that tumor cells were arraged for papillary with branches.Fiberous axis often contained rich small blood vessels.Some cases had expanded thyroid follicular structures with varying sizes which contained eosinophilic staining gelatinous substance around scallop-like secretory vacuoles,and a few of follicules contained light blue staining mucoid.Some had cystic-wall-like structure which included papillary and glandular structure.Some showed glandular arrangement.Individual case showed nest and adenoid cystic stucture.Major were coated simple cuboidal epithelium,minority for tabular and or columnar.Cytoplasm was clear and or light red.Cell nucleus was oval with fine and smooth chromatin.Nucleolus was not obvious with small one for minority.The tumor tissue was easy to note fresh and old bleeding.About a half of patients had necrostic bone in tumors.The tumors destroyed surrounding bone in some cases.There were fiberous hyperplasia with hyaline degeneration with some histocytes and lymphocytes infiltration in some patients.Calcification and gravel body were seen in a proportion of cases.Main clinical manifestations were partial or total hearing loss for all of patients,followed by the performance of tinnitus,with different degrees of cranial nerve damage.2 cases relapsed after one year and 7 years,respectively.Conclusion All of the patients show slow progress without death,and 2 cases relapse.