Although
pheochromocytoma is an uncommon cause of secondary
hypertension, it is also a curable form of
hypertension. With proper treatment, the outlook for patients with
pheochromocytoma is excellent. If undiagnosed or untreated,
pheochromocytoma causes serious complications and death. The key to reaching the diagnosis is a high index of suspicion coupled with careful clinical evaluation and laboratory testing. Our ability to diagnose and localize
pheochromocytoma has improved vastly, owing largely to the modern biochemical and radiologic techniques. Despite this, a number of
pheochromocytomas remain undiagnosed only to be uncovered after a serious complication or at postmortem. The diverse manifestations and problems presented by patients with
pheochromocytoma can be managed only with combined medical and surgical expertise, with assistance from our colleagues from the radiology and anesthesiology disciplines. Although sporadic
pheochromocytoma is the most common form of presentation, the overall clinical picture may depend on the subset of affected individual--childhood, pregnancy, associated MEN, and
neurocutaneous syndromes. Fortunately, more than 90 per cent of all
pheochromocytomas originate in the adrenal gland, but the
tumor can occur at any site in the sympathetic chain. The spectrum of clinical manifestations is so wide that a
pheochromocytoma may mimic a variety of common disorders. It is as challenging to diagnose what is not a
pheochromocytoma as it is to confirm its diagnosis. Surgical removal of
pheochromocytoma is the treatment of choice. With proper preoperative medical management aimed at blocking the effects of
catecholamines, operative mortality should be close to zero. For patients with inoperable, malignant, recurrent, or multicentric
pheochromocytomas, chronic medical
therapy is indicated in the form of alpha- and/or beta-blockade or inhibition of
catecholamine synthesis with
alpha-methyl-para-tyrosine. An understanding of the pathophysiology and natural course is vital to the rational management of patients with
pheochromocytoma.