Pheochromocytoma presenting as middeldorpf tumor: case report

Pheochromocytoma is a tumor that characteristically arises in the adrenal medulla. Extra-adrenal tumors make up approximately 10 per cent of all pheochromocytomas, with those occurring in the organ of Zuckerkandl being the most common. Others may occur anywhere along the sympathetic chain, including a number of reported cases in the bladder. A case is reported in which the tumor occurred deep in the presacral pelvic area, retroperitoneal and retrorectal, a so-called Middeldorpf tumor. Its rare presentation and technical demand of its removal underscore the importance of preoperative and intraoperative medical and pharmacologic management.