Surgical Management of Endolymphatic Sac Tumors

Objective: The purposes of this study were to (1) review the cumulative experience of surgical management of endolympatic sac tumors (ELSTs) in the literature, (2) review the experience with ELST management at a tertiary referral-based otology-neurotology practice, and (3) present our approach to management.

Study Design: A retrospective review was conducted of patients at a referral-based otology-neurotology practice.

Methods: A review of all available records from the House Clinic of patients with ELSTs was performed. Presentation, diagnostic workup, microsurgical approach, and outcomes were reviewed. A literature review using MEDLINE of studies reporting surgical outcomes of patients with ELSTs was also performed.

Results: The retrospective chart review revealed 10 patients with available charts. Of these patients, 6 had been previously reported on and updated follow-up is presented. Furthermore, outcomes in 10 more patients had been previously reported from our institution. Grouping of these patients provides surgical outcomes on the largest group of mostly non-von Hippel-Lindau (vHL) patients with ELSTs in the literature. Patients most commonly presented with sensorineural hearing loss, tinnitus, and dizziness. A majority of patients presented with large tumors exhibiting significant destruction of surrounding structures, including the cochlea, vestibule, and internal auditory canal. Patients underwent microsurgical resection with the intent for complete resection.

Conclusions: Although the extensive nature of ELSTs at time of diagnosis often precludes hearing preservation, complete microsurgical resection can be achieved safely with preservation of facial function in the majority of cases.