- What Is It?
- Life Expectancy
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:
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:
- Anxiety/panic attacks
- Stomach pain
- Side pain
- Dizziness on standing caused by low blood pressure
- Retinal damage due to high blood pressure
- Weight loss
- Paleness (pallor)
- 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:
How Is Pheochromocytoma Diagnosed?
Tests used to diagnose pheochromocytoma include:
- Plasma metanephrine testing
- 24-hour urine collection for catecholamines and metanephrines
- Imaging studies
- 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:
- 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:
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
- Kidney failure
- Nerve damage in the eyes
What Is the Life Expectancy for Pheochromocytoma?
Pheochromocytomas are rarely cancerous so for the most part, life expectancy is normal once a tumor is removed.
If pheochromocytoma is not diagnosed and treated promptly and the tumor is cancerous, the survival rate is diminished.