Abstract
von Hippel–Lindau (VHL) disease is a hereditary phakomatosis, a rare autosomal dominant neurocutaneous dysplasia complex with 80–100 % penetrance and variable delayed expressivity. It is characterised by visceral cysts and benign tumours in multiple organ systems, with potential for malignant change. Sex distribution is equal, and 20 % of cases are familial.
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Suggested Reading
Leung RS, Biswas SV, Duncan M et al. (2008) Imaging features of von Hippel-Lindau disease. Radiographics. 28 (1): 65–79
Taouli B, Ghouadni M, Corréas JM et al. (2003) Spectrum of abdominal imaging findings in von Hippel-Lindau disease. AJR Am J Roentgenol.181 (4): 1049–54.
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Zamboni, G., Gourtsoyianni, S. (2015). V. In: MDCT and MRI of the Liver, Bile Ducts and Pancreas. A-Z Notes in Radiological Practice and Reporting. Springer, Milano. https://doi.org/10.1007/978-88-470-5720-3_22
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DOI: https://doi.org/10.1007/978-88-470-5720-3_22
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