Retroperitoneal pheochromocytomas are very uncommon tumors. During the last 10 years only 3 cases have been operated on in our clinic. Two of them were primary retroperitoneal paragangliomas. These two patients are now well and without recurrence 1 and 10 years respectively after operation. The third patient had retroperitoneal metastases of an adrenal pheochromocytoma. Now, after 1 year he has disseminated metastases without response to therapy with (131I)metaiodobenzylguanidine (MIBG). The surgical procedure includes often extended resection and replacement of retroperitoneal blood vessels and requires therefore an experienced surgical team and optimal anesthetic management (Swan-Ganz-katheter). In two cases the aorta was resected and replaced with a Dacron-graft. Reimplantation of both renal arteries into the graft was necessary in one case. Further interventions were: nephrectomy (2x), resection and ligation of the inferior vena cava (1x), resection and replacement of the left renal vein (1x).

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