Endolymphatic Sac Tumor: A Report of 3 Cases and Discussion of Management

Abstract

Patients with an endolymphatic sac tumor (ELST) typically present with palsy of cranial nerves VII and/or VIII; other presenting symptoms include hearing loss, otalgia, occipital headaches, cranial nerve palsies, vertigo, gait ataxia, tinnitus, and otorrhea. ELSTs are extremely vascular, and they can invade and destroy temporal bone. Because of these characteristics, they are often mistaken for glomus tumors of the skull base. We describe the clinical presentation, evaluation, and management of ELSTs based on our review of the limited literature and our experience with 3 adults who presented to our tertiary care referral center with large ELSTs. Although these patients presented late in the course of their disease, their symptoms were relatively minor. Preoperative tumor embolization was performed, and a near-complete resection was achieved via an extended transotic approach in all 3 patients. The facial nerve was preserved without transposition in the first patient, the second patient underwent a primary nerve anastomosis, and the third required a cable graft of the facial nerve. Postoperative radiation therapy was administered to 2 of these patients. Follow-up by MRI detected no evidence of recurrence in any of the 3 patients.