Management of endolymphatic sac tumors: a case series report

Objective To study clinical characteristics of endolymphatic sac tumor (ELST) and its diagnosis and treatment. Methods ELST was diagnosed in 6 cases based on surgical and histological findings. These cases were reviewed for their clinical manifestations, differential diagnosis and surgical treatment techniques. Results There were 1 male and 5 females in this group, aged from 28 to 59 years (mean age=38.7 years). The tumor was in left ear in 4 cases and in right ear in the other 2 cases. Disease courses ranged from 5 to 30 years (mean duration= 12.6 years). Clinical presentations included sensorineural hearing loss (n=4), otorrhea and tinnitus (n=2), tinnitus and facial spasm(n=1), otorrhea with facial paralysis(n=l), and hearing loss with tinnitus (n=2). None of the cas-es was diagnosed as ELST preoperatively. Two cases were misdiagnosed as glomus jugulare tumor, 2 as chronic suppurative otitis media, 1 as sweat gland adenoma on biopsy and 1 as temporal bone tumor. Tumors were sprgi-tally resected in all 6 cases via the mastoidectomy (n=2) or combined oto-cervical or cranio-oto-cervical ap-proaches(n=4). Postoperative cerebrospinal fluid otorrhea occurred in 1 case. The tumors were confirmed on histo-logical examination to be a low-grade adenocarcinoma. All patients have survived at the time of this paper. Conclusion ELST is rare and commonly misdiagnosed and inadequately treated. Its prognosis is relatively favor-able because of its slow growth rate.