Endolymphatic sac tumor: a rare neoplasm of the vestibular system
DOI:
https://doi.org/10.18203/issn.2454-5929.ijohns20260073Keywords:
Endolymphatic sac tumor, Meniere’s syndrome, Translabyrinthine surgical removalAbstract
Endolymphatic sac tumors (ELSTs) are rare neoplasms originating from the endolymphatic sac, often associated with von Hippel-Lindau (VHL) disease. These tumors typically present with progressive hearing loss, vertigo, and vestibular symptoms, often mimicking Meniere’s disease. This case report discusses a 68-year-old female with a six-month history of progressive hearing loss and vertigo. Imaging revealed an ELST, and the patient underwent surgical resection via the translabyrinthine approach due to compromised hearing. Postoperatively, the patient underwent radiotherapy due to residual unresected tumor, stuck to posterior fossa dura and lying close to the ICA. A review of current literature on the diagnosis, treatment, and management strategies for ELSTs is also discussed.
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Raghunandhan S, Krishnan PV, Murali S, Kumar RS, Kameswaran M. Endolymphatic sac tumour: A neoplastic cause for Meniere’s syndrome. Indian J Otolaryngol Head Neck Surg. 2014;66(1):352–5. DOI: https://doi.org/10.1007/s12070-011-0395-3
Zanoletti E, Girasoli L, Borsetto D, Opocher G, Mazzoni A, Martini A, et al. Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies. Acta Otorhinolaryngol Ital. 2017;37(5):423–9. DOI: https://doi.org/10.14639/0392-100X-1402
Bambakidis NC, Megerian CA, Ratcheson RA. Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status. Otol Neurotol. 2004;25(5):773–81. DOI: https://doi.org/10.1097/00129492-200409000-00021
Yilmaz I, Bolat F, Demirhan B, Aydin V, Ozluoglu LN. Endolymphatic sac papillary tumor: a case report and review. Auris Nasus Larynx. 2008;35(2):276-81. DOI: https://doi.org/10.1016/j.anl.2007.06.009
Kim HJ, Hagan M, Butman JA, Baggenstos M, Brewer C, Zalewski C, et al. Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications. Laryngoscope. 2012;123(2):477-83. DOI: https://doi.org/10.1002/lary.23646
Mendenhall WM, Suárez C, Skálová A, Strojan P, Triantafyllou A, Devaney KO, et al. Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther. 2018;35(7):887–98. DOI: https://doi.org/10.1007/s12325-018-0730-0
Wick CC, Manzoor NF, Semaan MT, Megerian CF. Endolymphatic Sac Tumors. Otolaryngol Clin North Am. 2015;48(2):317-30. DOI: https://doi.org/10.1016/j.otc.2014.12.006
Gupta R, Vaidhyswaran AN, Murali V, Kameswaran M. Endolymphatic sac papillary carcinoma treated with surgery and post-operative intensity-modulated radiotherapy: A rare case report. J Cancer Res Ther. 2010;6(4):540–2. DOI: https://doi.org/10.4103/0973-1482.77062
Gioacchini FM, Chiarella G, Kaleci S, Iannella G, Viola P, Pisani D, et al. Surgical management of endolymphatic sac tumor: A systematic review and meta-analysis. J Int Adv Otol. 2023;19(3):248–54. DOI: https://doi.org/10.5152/iao.2023.22957
Moffat DA, Ransley PG, Preston PR. Donaldson's line: a surgical landmark in mastoid surgery. J Laryngol Otol. 1987;101(5):484-7.
Jackler RK, Cho KJ, Pitts LH. Endolymphatic sac tumor: a rare neoplasm associated with von Hippel-Lindau disease. Arch Otolaryngol Head Neck Surg. 1989;115(9):1063-7.
Ong CA, McLean CA, Kaye AH, et al. Immunohistochemical profile of endolymphatic sac tumors. Histopathology. 2005;46(1):94-100.
Hamilton BE, Crane GM, Pappas DG Jr, et al. Endolymphatic sac tumor: a clinicopathologic and immunohistochemical study of 8 cases. Ann Diagn Pathol. 2015;19(3):145-50.
Lindberg M, Engström B, Kindblom LG, et al. Endolymphatic sac tumor: a clinicopathologic and immunohistochemical study of two cases. Hum Pathol. 1994 Oct;25(10):1130-4.
Robertson JH, Robertson PW, Weiss LM, et al. Endolymphatic sac tumor: an immunohistochemical study of a rare tumor. Mod Pathol. 1994 Jan;7(1):89-93.
Li F, Zhang Y, Li W, Wu Q, Kong D, Shi X, et al. Grading system and surgical approaches for endolymphatic sac tumors. Eur Arch Otorhinolaryngol. 2021;278(5):1345–53. DOI: https://doi.org/10.1007/s00405-020-06185-3
Tang JD, Grady AJ, Nickel CJ, Ryan LE, Malone A, Canvasser L, et al. Systematic review of endolymphatic sac tumor treatment and outcomes. Otolaryngol Head Neck Surg. 2023;168(3):282–90. DOI: https://doi.org/10.1177/01945998221108313
Zhang Y, Li W, Li F, Wu Q, Kong D, Shi X, et al. Endolymphatic sac tumor: case report and literature review. Chin J Ophthalmol Otorhinolaryngol. 2020;22(3):242–247.
Wick CC, Manzoor NF, Semaan MT, Megerian CA. Endolymphatic sac tumor: case report and review of the literature. Ear Nose Throat J. 2020;99(4):158–161.
Reijneveld JC, Hanlo PW, Groenewoud G, Jansen G, van Overbeeke K, Tulleken. Endolymphatic sac tumor: A case report and review of the literature. Surg Neurol. 1997;48(4):368–73. DOI: https://doi.org/10.1016/S0090-3019(96)00553-8