PAPILLARY NEOPLASM OF THE ENDOLYMPHATIC SAC

A papillary neoplasm that was presumed to originate from the endolymph atic sac was described by Heffner in 1989. This tumor was considered t o be a ''low-grade adenocarcinoma'' because of its behavior: slow grow th, local destruction and failure to metastasize. The clinical manifes tations are hearing loss, vertigo, facial nerve paralysis and/or cereb ellar disorders. Endolymphatic sac tumors have previously been mistake n for such neoplasms as paraganglioma, choroid plexus papilloma and ca rcinoma, adenomatous tumor of the middle ear and secondary metastases. The diagnosis of this neoplasm is facilitated by CT and MRI. The trea tment of choice is total removal of tumor as soon as possible and requ ires clinical awareness of this rare but important pathologic entity.