Endolymphatic sac tumours

Research output: Contribution to journalReview articleContributedpeer-review

Contributors

  • G. Kempermann - , Neurozentrum, Salk Institute for Biological Studies (Author)
  • H. P.H. Neumann - , University Medical Center Freiburg (Author)
  • B. Volk - , Neurozentrum (Author)

Abstract

This review article surveys clinical and pathological literature on endolymphatic sac tumours (ELST) and summarizes characteristics that describe the entity. ELST are rare neuroectodermal neoplasms in the petrous bone, originating from inner ear structures. They can be encountered sporadically or in von Hippel-Lindau disease. The most prominent symptom is sensorineural deafness. Historically, nomenclature of invasive adenoid tumours in the petrous bone has been divergent, the term papillary adenocarcinoma used most frequently. Histologically, they have a follicular or papillary and adenoid pattern that can be easily confused with various other neoplastic conditions including metastatic carcinoma. It remains to be verified whether similar tumours (papillary adenocarcinomas) can originate from the middle ear. Middle ear adenomas have a similar appearance but probably originate from neural crest cells in the middle ear. ELST can express a variety of epitopes (including cytokeratin and neuroectodermal markers) which can be detected immunohistochemically. In cases of yon Hippel-Lindau disease the cerebellopontine angle should be included in routine radiological examinations to detect ELST before the tumours lead to deafness. In apparently sporadic cases of ELST, genetic testing for von Hippel-Lindau disease should be considered. Correct distinction of ELST from metastatic carcinoma prevents futile searches for unknown primary tumours.

Details

Original languageEnglish
Pages (from-to)2-10
Number of pages9
JournalHistopathology
Volume33
Issue number1
Publication statusPublished - 1998
Peer-reviewedYes
Externally publishedYes

External IDs

PubMed 9726042
ORCID /0000-0002-5304-4061/work/152544187

Keywords

Sustainable Development Goals

Keywords

  • Cancer, Deafness, Endolymphatic sac, Human, Skull base, Temporal bone, Tumour