Von Hippel-Lindau disease: delayed presentation as a cerebellar haemangioblastoma in an elderly patient.
نویسندگان
چکیده
Cerebellar haemangioblastomas are rare in the elderly. Those associated with the von Hippel-Lindau (VHL) disease, usually present during the third decade of life. We report an unusually delayed presentation in an 80 year old obligate carrier of VHL gene mutation.
منابع مشابه
Statistical analysis of the two stage mutation model in von Hippel-Lindau disease, and in sporadic cerebellar haemangioblastoma and renal cell carcinoma.
Analysis of the age incidence curves for unilateral and bilateral retinoblastoma led Knudson to propose that hereditary tumours may arise by a single event and sporadic tumours by a two stage mutation process. It has been suggested recently that sporadic renal cell carcinoma may arise from a two stage mutation process. We analysed the age incidence curves for symptomatic renal cell carcinoma (n...
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OBJECTIVES Haemangioblastoma of the CNS occurs as a sporadic entity and as a manifestation of the autosomal dominant von Hippel-Lindau disease with the major additional components retinal angioma, renal cancer, and pheochromocytoma. Genetic testing for germline mutations predisposing to von Hippel-Lindau disease has been available since identification of the VHL tumour suppressor gene. The impa...
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Haemangioblastoma, a benign vascular tumor derived from the capillary of endothelial cells, is an inherited disorder due to an autosomal dominant trait. The prevalence rates accounted for 1% to 2.5% of all intracranial tumors and 2% to 3% of all intramedullary spinal cord tumors. We report the infrequent case of a robust allegation of von Hippel-Lindau (VHL) associated haemangioblastoma in the ...
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Objective: To present an interesting case of pre-operative embolization of a cerebellar haemangioblastoma. Clinical Presentation and Intervention: A 36-year-old male presented with gradual, progressive headache and a positive family history of von Hippel-Lindau syndrome. MRI of the brain revealed a right cerebellar solid mass and cerebral angiography demonstrated its extensive hypervascular nat...
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ورودعنوان ژورنال:
- British journal of neurosurgery
دوره 23 1 شماره
صفحات -
تاریخ انتشار 2009