Endolymphatic sac tumor: a case report

Papillary tumors of the temporal bone are aggressive neoplasms which may oc cur sporadically or as a part of von Hippel-Lindau disease. The term 'endol ymphatic sac tumor' identifies the origin of these rare tumors. The clinica l manifestations are sensorineural hearing loss, facial paralysis, cerebell ar disorders and vertigo. The tumor is locally invasive, destructive and hy pervascular exhibiting consistent imaging and histopathologic features. The treatment of choice is the total removal of the lesion although complete e xcision of the advanced lesion is nearly impossible due to the anatomic com plexity of the endolymphatic sac and distinct patterns of extension. We pre sent a 50-year-old male patient with endolymphatic sac tumor with left side d sensorineural hearing loss and review the literature. (C) 2001 Elsevier S cience Ireland Ltd. All rights reserved.