Endolymphatic sac tumor: unique features of two cases and review of the literature.

Abstract

Endolymphatic sac tumors (EST) are rare intracranial tumors originating from the pars rugosa of the endolymphatic sac. Although typically described as histologically nonaggressive lesions, nevertheless they are termed adenocarcinomas and often become locally invasive. We report two patients with histologically proven EST with unique clinical features: the first pediatric case of an EST in an 11-year-old patient whose complaints started at the age of seven; and, a second patient, a 43-year-old man, the first report of metastatic EST which appeared in a remote location from the original site of surgery. Both patients underwent gamma-knife radiosurgery for recurrent tumor. This treatment has not been described previously for these tumors. Both patients have a follow-up of 7 years. Although not disease free they remain neurologically stable. We review the literature on EST.