Sporadic endolymphatic sac tumor : its clinical, radiological, and histological features, management, and follow-up

BACKGROUND: Sporadic endolymphatic sac tumor (ELST) is rare. We described the clinical, radiological, and histological features, treatment, and follow-up of ELST. METHOD: This was a retrospective analysis of 7 cases of sporadic ELST that were managed between 1993 and 2010. RESULTS: Twenty-five to 75 years was the age range of the patients. Subjective hearing loss and tinnitus were the most common presenting features. Five patients had total deafness and 2 had severe sensorineural hearing loss. The most common radiological feature was temporal bone destruction with tumor extension to cerebellopontine angle and posterior cranial fossa. Cholesterol or hemosiderin cysts around the tumor could be a characteristic feature. Major skull base procedures were performed in all 7 cases, and complete tumor excision was achieved in 6 of them. One patient needed a second surgery after she was referred to us after an incomplete first surgery. Recurrences were detected in 2 patients during follow-up; 1 of them received irradiation without minimal change to the tumor size and the second refused any treatment for the recurrence. Both of them are alive with disease. CONCLUSION: Early detection and radical surgical excision at first attempt give best results. Radiotherapy could be considered only in unresectable recurrences. © 2012 Wiley Periodicals, Inc. Head Neck, 2012.