Endolymphatic sac tumor: a rare tumor of internal ear. Report of two cases

Papillary armors of the temporal bone are rare and aggressive neoplasms. Re cently described, these tumors had initially a presumed middle-ear origin. Only recently,. convincing anatomic, morphological and immunohistochemical arguments exist for an endolymphatic sac origin (inner-ear origin). We repo rt two cases of endolymphatic sac tumor. These tumors can be encountered sp oradically or in Van Hippel-Lindau disease. They classically grow very slow ly, resulting in late clinical manifestations with expansive mass invading temporal bone and extending in posterior fossa. Radiologically, these endol ymphatic snc tumors can mimic metastatic carcinoma paraganglioma, or cerebe llar haemangioblastoma specially in von Hippel-Lindau disease. Histology sh ows a papillary epithelial tumor with hypervascular stroma without atypia. The treatment for these tumors is surgical and curative when early diagnose d. In apparently sporadic cases, genetic analysis for Von Hippel-Lindau dis ease should be considered.