Abstract
Objective: Endolymphatic sac tumor (ELST) is a low grade adenocarcinoma arising sporadically or associated with Von-Hippel-Lindau (VHL) disease. The tumor is locally aggressive to adjacent structures. Clinical manifestations depend on the extent of the disease. Treatment is surgical radical removal trough transpetrous approaches. When part of VHL the tumor is more aggressive and has higher incidence. We discuss the surgical therapy, the outcome of the VHL related to ELST tumors, with the aim of investigating the best option in the frame of a multi-organ disease.
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