Pheochromocytoma and functional paraganglioma.

Overview

PURPOSE OF REVIEW: Pheochromocytoma is a rare, but clinically important tumor of chromaffin cells. Advances in our understanding of the genetic alterations causing hereditary forms and the increasing sensitivity of biochemical assays allow for early identification of high risk individuals and families. Surgical intervention remains the treatment of choice for patients with pheochromocytoma. This article reviews recent developments in the diagnosis, treatment, and pathophysiology of pheochromocytoma, with the objective of developing new guidelines in the identification and management of the disease. It emphasizes current diagnostic and surgical approaches and discusses the potential for future developments in the field. SUMMARY: Advances in the molecular basis of pheochromocytoma have introduced new diagnostic modalities. Refinements in imaging techniques have improved the rate of detection of metastatic disease. Innovations in surgical techniques and trials of adrenal sparing surgery may find a niche in the surgical armamentarium.