Pheochromocytoma Signs and Symptoms
Introduction
- Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells
- Adrenal medulla is the most common site of origin (approximately 90%)
- However, tumors may develop anywhere along the abdominal sympathetic chain & are likely to be located near the aorta at the level of the inferior mesenteric artery or at its bifurcation
Etiology
- Most cases of pheochromocytoma have no known cause
- In a substantial minority of cases, genetic abnormalities have been identified in some familial forms of pheochromocytoma, as well as those associated with MEN2A and MEN2B, von Hippel-Lindau disease, neurofibromatosis type 1, & paraganglioma syndromes
Signs and Symptoms
- Pheochromocytoma can occur at any age
- Usually occurs during young to mid-adult life
- 10% of patients are asymptomatic
Majority of patients present w/ ≥1 of the following:
- Hypertension resistant to standard antihypertensive treatment
- May be associated w/ headaches, excessive sweating &/or palpitations
- Hypertensive crisis w/ malignant hypertension, hypertensive encephalopathy, aortic dissection or myocardial infarction (MI)
- Paroxysmal symptoms which suggest seizure disorder, anxiety attacks or hyperventilation
- Occurs in half of patients
- Usually sudden in onset but variable in length (eg min-several hr)
- Associated w/ headache, sweating, palpitations, apprehension, chest or abdomen pain, N/V
- Pallor may occur
- Very high BP usually w/ tachycardia
Other Symptoms
- Increased metabolic rate
- Mild-moderate wt loss
- Orthostatic hypotension
- Results from decreased plasma volume & dulled sympathetic reflexes
- Cardiac symptoms
- Sinus tachycardia, sinus bradycardia & other arrhythmias
- Acute MI or angina may occur even in the absence of coronary artery disease
- ECG changes
- Cardiomyopathy
- Multi-organ system failure w/ noncardiogenic pulmonary edema
- Carbohydrate intolerance
- Elevated hematocrit secondary to decreased plasma volume
- Hypercalcemia
- Fever, elevated erythrocyte sedimentation rate (ESR)
- Polyuria, occasionally rhabdomyolysis w/ myoglobinuric renal failure
- Hypokalemia