Bilateral pheochromocytoma in Von Hippel-Lindau syndrome simultaneously removed by lateral retroperitoneal endoscopic approach

Abstract

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant familial neoplastic syndrome caused by mutation in VHL tumor suppressor gene localized on chromosome 3p25. The disease is characterized by abnormal vascular proliferation and increased risk of developing renal cell carcinoma, pheochromocytoma, hemangioblastoma of the central nervous system, tumors of the endolymphatic bag, cysts of the kidney, liver and pancreas, epididymal cystadenomas, neuroendocrine tumors of the pancreas, angiomas in the retina. We report a case of a bilateral pheochromocytoma, simultaneously removed by unilateral total and contralateral subtotal retroperitoneal endoscopic adrenalectomy.