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von Hippel–Lindau syndrome


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A dominantly inherited familial cancer syndrome caused by mutation in the gene encoding the von Hippel–Lindau tumour suppressor protein. The syndrome involves a predisposition to a variety of malignant and benign neoplasms, most frequently retinal, cerebellar, and spinal haemangioblastoma, renal cell carcinoma (RCC), phaeochromocytoma, and pancreatic tumours. The highly vascular tumours associated with von Hippel–Lindau syndrome overproduce angiogenic peptides such as vascular endothelial growth factor (VEGF).

Subjects: Medicine and Health.


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