Background. Von Hippel-Lindau syndrome is a rare autosomal dominant disorder caused by genetic mutations in a tumor suppressor gene VHL. It is characterized by formation of multiple benign and malignant tumors, such as retinal and central nervous system hemangioblastomas, clear cell renal carcinomas, pheochromocytomas, pancre atic neuroendocrine tumors, as well as cysts in multiple organs in young age. Surgery is the method of choice for treatment of hormone-producing tumors, however situation becomes complicated if the procedure is applied during pregnancy.
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