Endolymphatic sac tumor at the cerebellopontine angle: A case report and review of literature

INTRODUCTION: Endolymphatic sac tumors may present as sporadic or may be associated with Von Hippel-Lindau disease. Patients generally present with hearing loss, tinnitus and vertigo. The tumor is highly vascular which may lead to erosion of the adjacent bony and vascular structures, resulting in heavy bleeding during surgery. PRESENTATION: A twenty-five year-old female presented with a five year history of chronic ear discharge, left sided facial weakness, and recent onset of ataxia. DISCUSSION: The unusual clinical presentation made management challenging, in large part due to profuse bleeding. Pre-operative embolization of the vessels supplying the tumor may reduce blood loss during surgical excision. Radiotherapy could be considered for any residual tumor. CONCLUSION: The patient was diagnosed with an endolymphatic sac tumor of sporadic origin which presented at the cerebellopontine angle and was managed with a multidisciplinary approach.