Low-grade papillary adenomatous tumors of the temporal bone: report of two cases and review of the literature.

Abstract

Two cases of tumors of the posterior portion of the petrous bone are presented. Both resulted in significant loss of hearing and caused extensive bone destruction. Microscopically, they corresponded to papillary adenomatous tumors with few histologically aggressive features. Immunohistochemical studies were done, and a positive reaction was obtained with the antibodies against the cytokeratins, Leu-7 and neuronal-specific enolase. A diagnosis of adenocarcinoma of the endolymphatic sac was made. These are rare tumors known for their long clinical prodrome and their local aggressiveness. Increased awareness will allow for earlier diagnosis and treatment.