Clinical features of pheochromocytoma and paraganglioma

The clinical features of pheochromocytoma are principally the result of catecholamine release from the tumor. As a consequence alarming, and occasionally spectacular, clinical presentations may occur. Hypertension is the most common manifestation of pheochromocytoma occurring in over 90% of patients. In about 10% the tumor is found during family screening or incidentally noted on imaging for another indication. The typical paroxysm or spell is the most characteristic manifestation, occurring in about 50% of patients. Headache, sweating, and palpitations are the most common symptoms noted during a paroxysm, but chest or abdominal pain is common as well. Although it is not necessary nor desirable to screen all hypertensive patients for pheochromocytoma, certain distinctive features warrant ruling out a chromaffin tumor: “spells,” new carbohydrate intolerance, orthostatic hypotension in the untreated state, malignant hypertension, recent onset hypertension, marked BP lability, Takotsubo cardiomyopathy, adrenal mass on imaging, unanticipated swings in BP in response to drugs or instrumentation, and family history of syndromic pheochromocytoma. The trick is to think of it; once pheochromocytoma is considered, the diagnosis can be readily confirmed or excluded in almost all cases.