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

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

J Laryngol Otol. 2017 Sep 06;:1-4

Authors: Bellairs JA, Gluth MB

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.

PMID: 28874214 [PubMed - as supplied by publisher]

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