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Endocrinology

Pheochromocytoma: Current Approaches and Future Directions

^aSection of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA; ^bEndocrine Surgical Unit, University of Sydney, ^cCancer Genetics, Kolling Institute of Medical Research, and ^dDepartment of Endocrinology, University of Sydney, Royal North Shore Hospital, New South Wales, Australia

Key Words. Pheochromocytoma • Management • Familial pheochromocytoma • Adrenal gland

Correspondence: Correspondence: Rebecca Sippel, M.D., H4/755 Clinical Science Center, 600 Highland Avenue, Madison, Wisconsin 53792, USA. Telephone: 608-263-1387; Fax: 608-263-7652; e-mail: sippel{at}surgery.wisc.edu

Received February 21, 2008; accepted for publication June 3, 2008; first published online in THE ONCOLOGIST Express on July 10, 2008.

Disclosure: H.C. has received honoraria from Novartis. The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors, planners, independent peer reviewers, or staff managers.

Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Because of the excess secretion of hormones, these tumors often cause debilitating symptoms and a poor quality of life. While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure. Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor. In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease.