What is pheochromocytoma?
A pheochromocytoma is a rare tumor that forms in the adrenal glands. The adrenal glands sit on top of the kidneys. They make hormones that control body functions like heart rate and blood pressure.
If your child has pheochromocytoma, they can make too much of certain catecholamine hormones that are part of the body’s natural response to stress, especially epinephrine (adrenaline). This can cause increases in heart rate, blood pressure, and feelings of anxiety.
Your child may have one or more pheochromocytomas. These tumors can be benign (not cancer) or malignant (cancer).
The main treatment for pheochromocytoma is surgery. Metastatic pheochromocytoma, or cancer that has spread to other parts of the body, such as the digestive tract, kidneys, and thyroid gland, is harder to treat.
Even with successful surgery, pheochromocytoma can come back. Long-term monitoring is important to watch for recurrence.
Symptoms of pheochromocytoma
Signs and symptoms of pheochromocytoma are often related to too much epinephrine, the hormone that is responsible for the “fight or flight” or stress response. Symptoms can come and go, and may include:
- High blood pressure
- High blood sugar
- Sudden, severe headaches
- Sweating more than usual
- Feeling hot (flushing) or having trouble being in the heat
- Fast or racing heartbeat (tachycardia) and skipped heartbeat (palpitations)
- Pain in the lower chest or upper abdomen
- Nausea with or without vomiting
- Weight loss without trying
- Pale skin
- Feelings of fear or anxiety
- Changes in vision
- Frequent urination
- Thirsty more than usual
- Headache
- Feeling tired (fatigue)
- Confusion
Rarely, people with pheochromocytoma can develop diabetes. In serious cases, blood sugar levels can get very high, and harmful substances called ketones can build up in the blood. This can be life-threatening if not treated.
Symptoms of pheochromocytoma can occur in episodes or attacks. Certain things, like stress, medicines, certain foods, and caffeine, can sometimes make symptoms worse.
Some people have no symptoms. They may find out they have a tumor when they get an imaging test for another reason. Others have a variety of symptoms, which depend on the type, location, size, and number of tumors.
Risk factors for pheochromocytoma
In many cases, the cause of pheochromocytoma is unknown. In some cases, pheochromocytoma can be caused by a gene change (mutation) that changes how cells function and grow. Several different gene mutations can cause pheochromocytoma.
In about 1 in 3 cases, the mutation is inherited or passed down from a person’s parents. People with certain genetic disorders and genetic predisposition syndromes may have a higher risk of pheochromocytoma tumors.
Some of these conditions include:
Sometimes, a person may have no known inherited genetic mutation leading to pheochromocytoma. This is called a sporadic pheochromocytoma.
Diagnosis of pheochromocytoma
Symptoms of pheochromocytoma can be like other health conditions, so it can be hard to diagnose. Tests to diagnose pheochromocytoma may include:
- Physical exam and health history to learn about symptoms, general health, past illness, and risk factors
- Blood and urine tests to check for catecholamines, hormones, and other substances. Your doctor may order a one-time or 24-hour urine collection test. It is important to collect all of the urine as instructed.
- Imaging tests such as ultrasound, CT, MRI, or PET Scan to check the size and location of the tumor. A specialized scan called an MIBG scan can show where neuroendocrine tumor cells are in the body.
- MIBG scan to take pictures of pheochromocytoma and other neuroendocrine tumors using a low dose of a radioactive tracer.
- Genetic counseling and testing for certain genetic predisposition syndromes, such as hereditary paraganglioma-pheochromocytoma syndrome. Your child may give a blood sample or have the inside of their cheek swabbed to get cells as part of genetic testing.
Ask your health care team how to prepare for any testing. Keep a record of symptoms and when they happen. This can help both you and your provider learn your child’s triggers so you can avoid them, if possible.
Some patients may also be at risk of a related tumor called paraganglioma. Paragangliomas develop outside the adrenal gland but can cause similar signs and symptoms to pheochromocytoma.
Treatment of pheochromocytoma
Because pheochromocytoma is a rare and complex type of tumor, it is important to get care from doctors who know about this condition and have treated it. Treatments for pheochromocytoma depend on several factors. These include:
- The tumor’s size and location
- If the tumor is a malignant (cancerous) pheochromocytoma
- Whether the cancer has spread
- If the tumor is new or has come back
Treatments may include:
- Medicines to control blood pressure, heart rate, or the effects of too much hormone in the body, especially before surgery
- Surgery to remove as much of the tumor as possible, and in some cases, the adrenal gland (adrenalectomy)
- MIBG therapy to treat tumors that cannot be completely removed, or that come back (reoccur)
- Chemotherapy to help treat pheochromocytoma that has spread
- Targeted therapies to treat cancer that has spread or come back
After treatment, your child may require medication to help manage blood pressure, heart rate, and other symptoms. Hormone replacement medicines may be needed if their adrenal glands were removed.
Pheochromocytoma crisis
Rarely, pheochromocytoma may cause a sudden, life-threatening condition called a pheochromocytoma crisis. Symptoms include dangerous changes in blood pressure and body temperature, difficulty thinking, or feeling very anxious. If not treated, it can lead to heart and other organ damage. If this happens, call 911 and seek medical help right away.
Prognosis for pheochromocytoma
If the tumor can be removed completely, the prognosis is usually good. However, pheochromocytoma can sometimes recur, so long-term monitoring may be needed.
Tumors that have spread or come back can be harder to treat. If this condition is not treated, there is a high risk for life-threatening problems such as high blood pressure that can lead to heart disease, organ damage, and stroke.
It is best to speak with your health care provider about your child’s prognosis.
Living with pheochromocytoma
Your child may need regular checkups and tests after their treatment. These may include:
- Regular physical exams
- Blood and urine tests
- Imaging tests
- Monitoring for problems with the central nervous system, skeletal system, or heart and blood vessels
If your child has an inherited syndrome that increases their risk of other health problems, they may need additional monitoring.
Your health care provider may recommend that your child wear a medical alert bracelet or ID that says they have pheochromocytoma. This helps health care providers know that your child has this rare condition in an emergency.
Help your child live a healthy lifestyle. Some things they can do are:
- Take all medicines as instructed.
- Eat a healthy diet as recommended by your care team.
- Watch for triggers that make your child’s condition worse. Your child may need to avoid food and drinks high in caffeine or a substance called tyramine, which can affect blood pressure. Tyramine is found in chocolate, fermented food, cheese, and some foods that have been dried or smoked.
- Learn how to manage stress using mindfulness and relaxation techniques.
- Get regular physical activity as recommended by your care team. Your child may need to avoid intense exercise that could trigger an attack.
- Have healthy sleep habits.
- Avoid alcohol use, smoking, and vaping.
Questions to ask your care team
