Objective: To study the clinnicopathological features and differential diagnosis of endolymphatic sac tumorfor improving the understanding of this rare tumor. Methods: Tumor specimen from a case of endolymphatic sac tumor was observed under light microscope. Immunohistochemistry examination was performed and relevant literatures were reviewed. Results: Histopathologic examination presented a papillary-glandular pattern, and was covered by a single layer columnar epithelium cells containing clear cytoplasm. There were minimal cellular atypia. The stroma was rich in large number of proliferative fibrous connective tissue. Immunohistochemically,the tumor cells were diffusely positive for AE1/AE3, EMA, vimentin, focally positive for GFAP, S-100, andnegative for Syn, CgA, NSE, TTF-1, TG. The proliferating index Ki-67 was about 1%. Conclusions: Endolymphatic sac tumor is rare, and lacks specific clinical manifestations. The pathological diagnosis depends on morphologic characteristics in paraffin-embedded tissue section and immunohistochemical staining. Whole intact tumor resection is the most effective treatment ofendolymphatic sac tumor.
HAN Dongyan, FU Huijun, HE Yanyan, XI Hao, WEI Qing
. Endolymphatic sac tumor: Clinicopathological features and review of literature[J]. Journal of Diagnostics Concepts & Practice, 2018
, 17(06)
: 711
-714
.
DOI: 10.16150/j.1671-2870.2018.06.016
[1] Joseph BV, Chacko G, Raghuram L, et al.Endolymphatic sac tumor: a rare cerebellopontine angle tumor[J]. Neurol India,2002,50(4):476-479.
[2] Hassard AD, Boudreau SF, Cron CC.Adenoma of the endolymphatic sac[J]. J Otolaryngol,1984,13(4):213-216.
[3] Li JC, Brackmann DE, Lo WW, et al.Reclassification of aggressive adenomatous mastoid neoplasms as endolymphatic sac tumors[J]. Laryngoscope,1993,103(12):1342-1348.
[4] Leon B, Eveson JW, Reichart P, et al.WHO classification of tumours. Pathology and genetics of head and neck tumours[M]. Lyon:IARC Press,2005:355-356.
[5] Wick CC, Manzoor NF, Semaan MT, et al.Endolympha-tic sac tumors[J]. Otolaryngol Clin North Am,2015,48(2):317-330.
[6] Zanoletti E, Girasoli L, Borsetto D, et al.Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies[J]. Acta Otorhinolaryngol Ital,2017,37(5):423-429.
[7] Bambakidis NC, Megerian CA, Ratcheson RA.Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status[J]. Otol Neurotol,2004,25(5):773-781.
[8] Schnack DT, Kiss K, Hansen S, et al.Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness[J]. J Int Adv Otol,2017,13(2):289-291.
[9] Jagannathan J, Butman JA, Lonser RR, et al.Endolymphatic sac tumor demonstrated by intralabyrinthine hemor-rhage. Case report[J]. J Neurosurg,2007,107(2):421-425.
[10] Manski TJ, Heffner DK, Glenn GM, et al.Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease[J]. JAMA,1997,277(18):1461-1466.
[11] 饶秋, 周晓军, 金行藻, 等. 内淋巴囊瘤的临床病理及分子遗传学研究二例[J]. 中华病理学杂志,2010,39(6):412-413.
[12] 苏钰, 申卫东, 王翠翠, 等. 并发于von Hippel-Lindau综合征的内淋巴囊肿瘤VHL基因突变检测[J]. 中华耳鼻咽喉头颈外科杂志,2013,48(11):913-918.
[13] Kim HJ, Hagan M, Butman JA, et al.Surgical resection of endolymphatic sac tumors in von Hippel-Lindau di-sease: findings, results, and indications[J]. Laryngoscope,2013,123(2):477-483.
[14] Bambakidis NC, Rodrigue T, Megerian CA, et al.Endolymphatic sac tumor metastatic to the spine. Case report[J]. J Neurosurg Spine,2005,3(1):68-70.
[15] Tay KY, Yu E, Kassel E.Spinal metastasis from endolymphatic sac tumor[J]. Am J Neuroradiol,2007, 28(4):613-614.
