Origin of Endolymphatic Sac Tumor

Abstract

Autopsy temporal bone sections showing a one mm papillary glandular neoplasm, confined to the left endolymphatic duct, are described. This is the second literature report confirming the post-mortem site of origin of the "endolymphatic sac tumor". The patient died after surgery for right vestibular schwannoma, but no features of von Hippel Lindau disease or neurofibromatosis 2 had been displayed clinically or at autopsy. A study of the epithelium of normal human mature endolymphatic ducts and sacs (EDSs) in archival temporal bone sections showed hyperplastic tubular outgrowths, usually situated in the intraosseous portion of the endolymphatic sac, in most cases. Such appearances imply that the epithelium of the EDS has the potential of producing a malignant papillary glandular neoplasm. Papillary ingrowths, some forming collagenous polypoid projections, and cysts were frequent among the epithelial cells. Psammoma bodies were present in the ducts and sacs of older patients. Appearances suggesting erosion of the bony interface of vestibular aqueduct with EDS could be ascribed to the entry and exit of blood vessels into and from the vestibular aqueduct. Care should be taken in the evaluation of surgical or autopsy material from EDSs not to overcall any of these normal features as malignant.