A Case of Endolymphatic Sac Tumor Removal with Modified Translabyrinthine Approach / 대한이비인후과학회지

ABSTRACT

Endolymphatic sac tumor (ELST) is a very rare tumor arising from the epithelium of the endolymphatic sac. Pathologically, ELST shows papillary growth patterns with extensive bony and soft tissue destruction. ELST commonly shows symptoms of endolymphatic hydrops such as hearing loss, vertigo and tinnitus by blocking the endolymphatic duct. As the tumor enlarges, patients experience various symptoms that include multiple cranial nerve palsy. Ten to thirty percents of ELST are related to Von Hippel Lindau (VHL) diseases. Also, bilateral tumors can develop when a patient has VHL-related ELST. The goal of treatment for ELST is total tumor eradication. The type of surgical approach is chosen by the surgeon in consideration of the tumor grade and the hearing status of the patient. In this report, we present a patient with ELST who was successfully treated with the modified translabyrinthine approach. We have also reviewed relevant literatures.