Pheochromocytoma – Current concepts in diagnosis and management

SummaryPheochromocytomas, tumors originating from chromaffin tissue, commonly present with symptoms and signs of catecholamine excess. They may be detected incidentally, as part of multiple endocrine neoplasia, or during unrelated surgery. The previously high mortality (25–40%) has been reduced to <2% primarily due to understanding of catecholamine physiology and adequate preoperative preparation. Advances in molecular biology, genomics, biochemistry and biotechnology often lead to pre-symptomatic diagnosis. Advanced imaging techniques have allowed accurate localization. Availability of an array of vasoactive drugs, monitoring and perioperative care has positively impacted the intra operative course. Finally, advent of laparoscopic and robotic adrenal-sparing adrenalectomy have resulted in reduced hospital stay, earlier oral intake and resumption of normal activity. Management strategies are evolving for the pregnant patient with pheochromocytoma, and the patient with a malignant pheochromocytoma.