Background. Hereditary paraganglioma-pheochromocytoma syndrome is characterized by paragangliomas (tumors that arise from neuroendocrine tissues symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines and are located in the lower mediastinum, abdomen, and pelvis.
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.
