Symptoms of Pheochromocytoma

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Most adult patients have paroxysmal symptoms, lasting minutes to hours, consisting of headache (80%), perspiration (70%), and palpitations (60%); other symptoms are commonly present, such as anxiety (50%), a sense of dread, tremor (40%, with epi-nephrine-secreting tumors), or paresthesias. Recurrent chest discomfort, abdominal pain, and vomiting are also frequent symptoms. The abdominal pain may be caused by ischemic enterocolitis. Sweating (initially palms, head, and shoulders) usually occurs. Drenching sweats can occur, even in a cool environment, usually as an attack subsides. The reflex eccrine sweating that occurs later in an attack is ther-

moregulatory, dissipating heat that was acquired during prolonged vasoconstriction during a paroxysm. Constipation is common, and toxic megacolon has rarely occurred. Many patients have visual changes during acute attacks. Paroxysms usually begin abruptly and subside slowly. The episodes may not recur for months or may recur many times daily. Each patient tends to have a particular pattern of symptoms, with the frequency or severity of episodes usually increasing over time. Attacks can occur without provocation or may occur with certain activities, such as bending, rolling over in bed, exertion, abdominal palpation, or micturition (with bladder paragangliomas). The interindividual variability in manifestations is striking; most patients have dramatic symptoms, but others with incidentally discovered secretory pheochromocytomas are completely asymptomatic. Patients who develop pheochromocytomas as part of multiple endocrine neoplasia (MEN) type II are especially prone to be normotensive and asymptomatic.

Children with pheochromocytoma are more prone to diaphoresis and visual changes than are adults. They are prone to have sustained (instead of paroxysmal) hypertension. Nausea, vomiting, headache, weight loss, polydipsia, polyuria, and convulsions occur frequently in children with pheochro-mocytoma. Children with pheochromocytoma may develop edema and erythema of the hands, a condition rarely found in adults.15 Children are more prone to having multiple tumors; in one series, 39% had bilateral adrenal pheochromocytomas, an adrenal pheochromocytoma plus a paraganglioma, or multiple paragangliomas; single paragangliomas were reported in an additional 14% of children.16

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