What Is Pheochromocytoma?
A pheochromocytoma is a rare, usually benign, tumor that can cause life-threatening high blood pressure (hypertension). The tumors produce too much catecholamine hormone [such as norepinephrine (noradrenaline), epinephrine (adrenaline), and dopamine] which can cause episodes of high blood pressure. In about 10% of cases, pheochromocytomas are malignant (cancerous) but they can be cured if they are surgically removed.
What Are Symptoms of Pheochromocytoma?
Pheochromocytoma spells typically include four main symptoms:
- Headaches
- Heart palpitations
- Excessive sweating
- Severe high blood pressure (hypertension)
These spells follow a pattern:
- Frequency of spells varies from several times daily to once a month
- The duration of spells varies from seconds to hours
- Over time, spells usually occur more often and increase in severity as the tumor grows
Other symptoms that may occur during a pheochromocytoma spell include:
- Tremor
- Nausea
- Weakness
- Anxiety/panic attacks
- Stomach pain
- Side pain
- Constipation
- Dizziness on standing caused by low blood pressure
- Retinal damage due to high blood pressure
- Weight loss
- Paleness (pallor)
- Fever
- Tremor
- Neurofibromas
- Fast, irregular heartbeats
- Excess fluid in the lungs (pulmonary edema)
- Heart muscle damage (cardiomyopathy)
- Disruption in movement of the bowel (ileus)
- Café au lait spots
What Causes Pheochromocytoma?
About one-third of cases of pheochromocytoma are caused by an inherited genetic mutation. The other two-thirds of cases do not have a known cause (idiopathic).
Pheochromocytoma spells may be caused by certain triggers, such as:
- Stress
- Physical activity
- Certain medications such as beta-blockers or anesthetics
- Surgery
- Childbirth
- Massage
- Pressure on the tumor
- Foods that contain large amounts of the amino acid tyramine, such as chocolate, cheese, and red wine
How Is Pheochromocytoma Diagnosed?
Tests used to diagnose pheochromocytoma include:
- Plasma metanephrine testing
- 24-hour urine collection for catecholamines and metanephrines
- Imaging studies
- Abdominal CT scans
- Magnetic resonance imaging (MRI)
- Scintigraphy
- PET scans
- Other studies to rule out other conditions that have similar symptoms:
- Blood tests
- Parathyroid hormone level
- Calcium level
- Screening for mutations in the ret proto-oncogene
- Genetic testing
- Consultation with an ophthalmologist to rule out retinal angiomas
- Blood tests
What Is the Treatment for Pheochromocytoma?
The preferred treatment for pheochromocytoma is surgical removal of the tumor, which usually cures the condition.
Pre-operatively, patients may need to take medications to reduce blood pressure to prepare for surgery, such as:
- Alpha-blockers
- Beta-blockers
- Calcium channel blockers
- Angiotensin receptor blockers (ARBs)
Patients may also be advised to consume a high salt diet prior to surgery.
Pheochromocytomas are usually not malignant (cancerous), but if they are, they may be treated with:
- Radiation
- Chemotherapy
- Antineoplastic medications
What Are Complications of Pheochromocytoma?
Complications of pheochromocytomas include:
- High blood pressure (hypertension) - most common complication
- Irregular heartbeats
- Inflammation of the heart muscle (myocarditis)
- Heart attack
- Enlarged heart that is unable to pump blood efficiently (dilated cardiomyopathy)
- Fluid in the lungs (pulmonary edema)
- Hypertensive encephalopathy
- Altered mental status
- Focal neurologic signs and symptoms
- Seizures
- Stroke
- Bleeding in the brain (intracerebral hemorrhage)
- Kidney failure
- Nerve damage in the eyes