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

作者：Bellairs J A; Gluth M B^*

来源：Journal of Laryngology and Otology, 2018, 132(1): 75-78.

DOI：10.1017/S0022215117001888

• Endolymphatic Sac
• Endolymphatic Hydrops
• Von Hippel-Lindau Disease
• Temporal Bone

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.

• 出版日期2018-1