ENDOLYMPHATIC SAC TUMORS - HISTOPATHOLOGIC CONFIRMATION, CLINICAL CHARACTERIZATION, AND IMPLICATION IN VON HIPPEL-LINDAU DISEASE

The term ''endolymphatic sac tumor'' (ELST) was coined to identify the likely origin of aggressive papillary tumors of the temporal bone. To evaluate the validity of this designation, the temporal bone collecti on at the Massachusetts Eye and Ear Infirmary was accessed in an effor t to determine the pathologic relationship between these tumors and th e endolymphatic sac. The search resulted in the identification of a de -novo papillary epithelial lesion arising within the confines of the e ndolymphatic sac in a patient with von Hippel-Lindau (VHL) disease who harbored a large, destructive ELST in the opposite temporal bone. Thi s finding provides the most substantial evidence to date regarding the origin of the ELST and the accuracy of its nomenclature. Seven additi onal clinical cases of ELST were identified and analyzed in order to d efine the natural history of these tumors. All patients had a history of sensorineural hearing loss diagnosed an average of 10.6 years prior to tumor discovery. The presence of a polypoid external auditory cana l mass, facial paralysis, and evidence of a destructive mass arising o n the posterior fossa surface of the temporal bone were common physica l and radiographic findings. The management of these patients, as well as those who are probably prone to such tumors (i.e., VHL patients), is discussed.