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

Papillary armors of the temporal bone are rare and aggressive neoplasms. Recently 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 report two cases of endolymphatic sac tumor. These tumors can be encountered sporadically or in Van Hippel-Lindau disease. They classically grow very slowly, resulting in late clinical manifestations with expansive mass invading temporal bone and extending in posterior fossa. Radiologically, these endolymphatic snc tumors can mimic metastatic carcinoma paraganglioma, or cerebellar haemangioblastoma specially in von Hippel-Lindau disease. Histology shows a papillary epithelial tumor with hypervascular stroma without atypia. The treatment for these tumors is surgical and curative when early diagnosed. In apparently sporadic cases, genetic analysis for Von Hippel-Lindau disease should be considered.