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A histopathological connection between a fatal endolymphatic sac tumour and von Hippel–Lindau disease from 1960

  • J A Bellairs (a1) (a2) and M B Gluth (a2)
Abstract Objective:

To highlight a case from 1960 connecting endolymphatic sac tumour and von Hippel–Lindau disease.

Case report:

In 1960, a 24-year-old woman presented with unilateral hearing loss, pulsatile tinnitus and a mass visible on otoscopy. The patient underwent surgical biopsy, which was complicated by haemorrhage, and ultimately resulted in death. At autopsy, a destructive temporal bone neoplasm with cystic and papillary architecture was observed that had eroded into the otic capsule. Intra-abdominal lesions consistent with von Hippel–Lindau disease were also observed, and the surgeon postulated a connection between endolymphatic sac tumour and von Hippel–Lindau disease.


A review of the literature was carried out using PubMed.


Endolymphatic sac tumours are rare neoplasms of the temporal bone that can occur sporadically or as part of von Hippel–Lindau disease. The connection between endolymphatic sac tumour and von Hippel–Lindau disease was first proposed in 1988 and formalised in 1997. We believe that this case represents the first documented connection between endolymphatic sac tumour and von Hippel–Lindau disease.

Corresponding author
Address for correspondence: Dr Michael B Gluth, Section of Otolaryngology – Head and Neck Surgery, University of Chicago Medical Center, 5841 S. Maryland Ave, MC 1035, Chicago, IL 60637, USA Fax: +1 773 702 6809 E-mail:
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12 Obituary: A. N. GRIFFITH, M.B., B.Chir., F.R.C.S., D.L.O. Br Med J 1961;2:1296
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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