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5 results

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

  • J A Bellairs, M B Gluth
  • Journal:
    The Journal of Laryngology & Otology / Volume 132 / Issue 1 / January 2018
    Published online by Cambridge University Press:
    06 September 2017, pp. 75-78
    Print publication:
    January 2018
    • Article
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  • 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.

  • 34 - ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY

  • from PART III: - VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 237-246

  • Summary

    Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder with a 95% penetrance at age 60 and incomplete expression that is characterized by the development of various benign and malignant tumors and cysts. Hemangioblastoma (HB), the most characteristic central nervous system (CNS) lesions, are highly vascular tumors comprising approximately 3% of all tumors of the CNS. HBs are diagnosed by magnetic resonance imaging (MRI) of the brain and the spinal cord. Presentation of HBs with a stroke syndrome is rare despite their highly vascular nature, and the risk of hemorrhage associated with them is not yet known. Screening for VHL disease following the diagnosis of a cerebral HB is indispensable given the high risk of associated lesions with hereditary forms. The best treatment for this tumor is surgical resection. If the tumor is difficult to remove from its primary site, it can sometimes be treated with radiosurgery.