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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.

Method:

A review of the literature was carried out using PubMed.

Conclusion:

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.

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Copyright
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: michaelgluth@mac.com
References
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1 Bailey, BJ, Johnson, JT, Newlands, SD. Head & Neck Surgery–Otolaryngology. Philadelphia: Lippincott Williams & Wilkins, 2006;3053
2 Hassard, AD, Boudreau, SF, Cron, CC. Adenoma of the endolymphatic sac. J Otolaryngol 1984;13:213–16
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4 Heffner, DK. Low-grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases. Cancer 1989;64:2292–302
5 Li, JC, Brackmann, DE, Lo, WW, Carberry, JN, House, JW. Reclassification of aggressive adenomatous mastoid neoplasms as endolymphatic sac tumors. Laryngoscope 1993;103:1342–8
6 Lonser, RR, Glenn, GM, Walther, M, Chew, EY, Libutti, SK, Linehan, WM et al. von Hippel-Lindau disease. Lancet 2003;361:2059–67
7 Eby, TL, Makek, MS, Fisch, U. Adenomas of the temporal bone. Ann Otol Rhinol Laryngol 1988;97:605–12
8 Manski, TJ, Heffner, DK, Glenn, GM, Patronas, NJ, Pikus, AT, Katz, D et al. Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA 1997;277:1461–6
9 Bausch, B, Wellner, U, Peyre, M, Boedeker, CC, Hes, FJ, Anglani, M et al. Characterization of endolymphatic sac tumors and von Hippel–Lindau disease in the International Endolymphatic Sac Tumor Registry. Head Neck 2016;38:E6739
10 Choo, D, Shotland, L, Mastroianni, M, Glenn, G, van Waes, C, Linehan, WM et al. Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg 2004;100:480–7
11 Bambakidis, NC, Megerian, CA, Ratcheson, RA. Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status. Otol Neurotol 2004;25:773–81
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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