Low-grade adenocarcinoma of endolymphatic sac mimicking jugular paraganglioma at clinical and neuroradiological examination.

We report a case of low-grade adenocarcinoma of endolymphatic sac origin mimicking jugular paraganglioma at clinical and neuroradiological examination. The lesion occurred in a 72-year-old male who presented with a long-standing history of right-sided hearing loss and a few-week history of progressive facial nerve palsy and right aural pain. At histology, the tumor was composed of pseudoglandular spaces with papillary infoldings. Lumina contained colloid-like material. The lesion was surgically removed with suboccipital approach following endoarterial embolization. This study emphasizes that low-grade adenocarcinomas of endolymphatic sac origin extending to posterior cranial fossa and jugular paraganglioma may be indistinguishable preoperatively at clinical and radiological levels.