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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Headache, palpitations, and sweating in conjunction with either hypertension (may be paroxysmal) or orthostatic hypotension

  • Pressor response to anesthesia induction or to antihypertensive or sympathomimetic drugs

  • Biochemical evidence of excess catecholamines

  • Adrenal tumor or tumor along the sympathetic chain (paraganglioma)

GENERAL CONSIDERATIONS

  • Pheochromocytoma is a catecholamine-producing tumor of the sympathoadrenal system that is usually sporadic in occurrence but can be part of a familial syndrome with multiple endocrine neoplasms

  • These tumors predominantly secrete norepinephrine, but about 15% secrete mainly epinephrine

  • Hypertension is the major manifestation of these tumors, which is sustained in the majority, but these tumors account for < 0.1% of underlying primary causes of hypertension

  • Episodic hypertension due to catecholamine release is the classic manifestation, but this occurs in only about one-third of patients

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Episodic palpitation

  • Headache

  • Sweating

  • Tremor

  • Pallor

PHYSICAL EXAM FINDINGS

  • Sustained or episodic hypertension

  • Orthostatic hypotension

  • Hyperventilation

  • Tachycardia

  • Café-au-lait spots

DIFFERENTIAL DIAGNOSIS

  • Other causes of hypertension, headaches, palpitations, and orthostatic hypotension

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • A plasma epinephrine and norepinephrine > 2000 pg/mL is diagnostic

  • Twenty-four-hour urine for metanephrines and free catecholamines

  • If plasma catecholamine 1000–2000 pg/mL, repeat 3 hours after clonidine 0.3 mg PO; a level > 500 pg/mL suggests pheochromocytoma

ELECTROCARDIOGRAM

  • Tachycardias, nonspecific ST-T changes, and prominent U waves are common

  • Less common are right and left bundle branch block

IMAGING STUDIES

  • CT or MRI of the abdomen to localize the tumor after a positive serum catecholamine study

  • Iodine-131 metaiodobenzylguanidine (MIBG) scan may help find tumors not seen by CT or MRI

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected pheochromocytoma

HOSPITALIZATION CRITERIA

  • Blood pressure > 220/120 mm Hg

  • Planned surgery

MEDICATIONS

  • Preoperative alpha blockers and then beta1-selective beta blockade

  • IV fluids to reverse volume depletion

  • Metyrosine for refractory patients who cannot undergo surgery

SURGERY

  • Surgical removal of the catecholamine-producing tumor(s)

MONITORING

  • ECG and blood pressure in hospital and during clonidine suppression test

DIET AND ACTIVITY

  • Low-sodium diet

  • Restriction of vigorous exercise until tumor is removed

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • After successful surgery

FOLLOW-UP

  • Routine postsurgical follow-up

COMPLICATIONS

  • Stroke

  • Heart failure

  • Aortic dissection

  • Myocardial infarction

  • Renal failure

  • Ventricular tachyarrhythmia and sudden death

PROGNOSIS

  • Excellent with successful removal of the ...

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