Published: 2021-09-27

Multicranial neuropathy secondary to endolymphatic sac tumor

Tanvir Hussain, Thomas J. Crotty, Eoin F. Cleere, Mel Corbett, Aishan Patil, Marcus Choo


Endolymphatic sac tumors (ELST) are rare neuroectodermal neoplasms arising from the epithelium of the endolymphatic sac or duct. Despite their benign histopathological features, ELSTs frequently demonstrate a locally destructive growth pattern with involvement of the skull base and cranial nerves. While ELSTs may arise sporadically, the majority of cases occur in association with Von Hippel-Lindau (VHL) syndrome. ELSTs are commonly diagnosed late due to their slow growing nature and non-specific symptomatology. Surgical resection is the treatment of choice. However, due to the location of these tumors in the lateral skull base surgical intervention carries a high risk of injury to critical neurovascular structures in close proximity. We presented the case of a 51 years old gentleman with a three-months history of hearing loss and otalgia. He subsequently developed multiple cranial neuropathies and was diagnosed with a sporadic ELST. He underwent a complete surgical resection and received adjuvant radiotherapy.  


Endolymphatic sac tumor, Von Hippel-Lindau disease, Head and neck oncology, Lateral skull base surgery

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Manski TJ, Heffner DK, Glenn GM, Patronas NJ, Pikus AT, Katz D, et al. Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA. 1997;277(18):1461-6.

Poletti AM, Dubey SP, Barbo R, Pericotti S, Fiamengo B, Colombo G, et al. Sporadic endolymphatic sac tumor: its clinical, radiological, and histological features, management, and follow-up. Head Neck. 2013;35(7):1043-7.

Gaffey MJ, Mills SE, Fechner RE, Intemann SR, Wick MR. Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma. Am J Surg Pathol. 1988;12(10):790-7.

Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer. 1989;64(11):2292-302.

Choo D, Shotland L, Mastroianni M, Glenn G, van Waes C, Linehan WM, Oldfield EH. Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg. 2004;100(3):480-7.

Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer. 1989;64(11):2292-302.

Li JC, Brackmann DE, Lo WW, Carberry JN, House JW. Reclassification of aggressive adenomatous mastoid neoplasms as endolymphatic sac tumors. Laryngoscope. 1993;103(12):1342-8.

Patel NP, Wiggins RH, Shelton C. The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 2006;116(1):40-6.

Kilickesmez O. Endolymphatic sac tumor in a patient with von Hippel-Lindau disease: MR imaging findings. Diagn Interv Radiol. 2006;12(1):14-6.

Krzysztolik K, Cybulski C, Sagan L, Nowacki P, Lubinski J. Endolymphatic sac tumours and von Hippel-Lindau disease - case report, molecular analysis and histopathological characterization. Folia Neuropathol. 2009;47(1):75-80.

Wick CC, Manzoor NF, Semaan MT, Megerian CA. Endolymphatic sac tumors. Otolaryngol Clin North Am. 2015;48(2):317-30.

Nevoux J, Nowak C, Vellin JF, Lepajolec C, Sterkers O, Richard S, Bobin S. Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol. 2014;35(5):899-904.

Poletti AM, Dubey SP, Colombo G, Cugini G, Mazzoni A. Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone. Rep Pract Oncol Radiother. 2016;21(4):391-4.

Sinclair G, Saffar Y, Brigui M, Martin H, Bystam J, Benmakhlouf H, et al. Gamma knife radiosurgery in the management of endolymphatic sac tumors. Surg Neurol Int. 2018;9:18.