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Abstract

Medicine

Surgical Treatment of an Endolymphatic Sac Tumor

Published: May 26th, 2023

DOI:

10.3791/65079

1Otolaryngology, Audiology and Phoniatrics Operative Unit, University of Pisa, 2Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, 3Neuroradiology Operative Unit, Pisa University Hospital, 4Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, 5Clinical Science, Intervention and Technology, Karolinska Institute, 6Department of Clinical and Experimental Medicine, University of Pisa

Abstract

Endolymphatic sac tumors (ELST) are low-grade papillary adenocarcinoma originating from the endolymphatic sac. Usually slow-growing, with local aggressiveness and a low risk of distant metastases, ELST can be sporadic but also frequently associated with von Hippel Lindau disease. The current treatment of ELST is primarily surgical resection. A 55-year-old woman accessed our otologic tertiary level referral center for a sudden worsening of hearing loss in her left ear and vertigo. A magnetic resonance (MRI) and computer tomography scan study subsequently showed a mass in the petrous bone; hence, the presence of an ELST was hypothesized. After embolization of the mass, the patient underwent surgical removal of the lesion. The resection of the mass was done through a translabirinthine approach, with an uneventful procedure. No residual disease remained after surgery. After 24 months of radiologic follow up with MRI, there are no signs of recurrence disease. This paper reports the management of this sporadic ELST, as well as the follow up results, providing clinicians this protocol for the handling of such a challenging otologic skull base surgery and rare disease.

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Keywords Endolymphatic Sac Tumor

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