[Papillary tumor of the endolymphatic sac]

A papillary neoplasm that was presumed to originate from the endolymphatic sac was described by Heffner in 1989. This tumor was considered to be a "low-grade adenocarcinoma" because of its behavior: slow growth, local destruction and failure to metastasize. The clinical manifestations are hearing loss, vertigo, facial nerve paralysis and/or cerebellar disorders. Endolymphatic sac tumors have previously been mistaken for such neoplasms as paraganglioma, choroid plexus papilloma and carcinoma, adenomatous tumor of the middle ear and secondary metastases. The diagnosis of this neoplasm is facilitated by CT and MRI. The treatment of choice is total removal of tumor as soon as possible and requires clinical awareness of this rare but important pathologic entity.