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. One of the tumors - pheochromocytoma in pregnancy in-creases mortality risk for foetus and mother therefore appropriate treatment should be chosen.
Material and methods. We are presenting a clinical case of a patient with already known VHL syndrome and single adrenal gland pheochromocytoma, who was followed without surgical treatment. When she got unplanned pregnancy, her blood pressure began to increase already at the first trimester. She was immediately preprared for a pheochromocytoma surgery and adrenalectomy was performed at the second trimester of pregnancy. Atfer the surgery, hormone replacement therapy was prescribed due to adrenal insufficiency. At 39 weeks healthy male neonate was born by caesarean section.
Conclusions. In pregnant women with VHL syndrome and pheochromocytoma, the adequate preparation for surgery, selection of the most appropriate time for procedure and prescription of effective adrenal replacement therapy is crucially important in order to achieve good results for mother and foetus.
