Pheochromocytoma

11 min read

Pheochromocytoma is a rare tumor of the adrenal glands that produces hormones called catecholamines. These hormones can cause a wide range of symptoms, including high blood pressure, headaches, and sweating. Pheochromocytomas are usually benign, but can be malignant in some cases. They are most commonly found in adults, but can occur in children as well.

Pheochromocytomas are usually diagnosed through a combination of medical imaging tests, such as CT scans and MRI scans, and blood tests. Treatment typically involves surgery to remove the tumor, and may also include medications to control blood pressure and other symptoms. In some cases, radiation therapy may be used to shrink the tumor.

Pheochromocytomas can be inherited, and are associated with several genetic syndromes, including multiple endocrine neoplasia type 2 (MEN2) and von Hippel-Lindau disease. People with a family history of pheochromocytoma should talk to their doctor about genetic testing and screening.

Symptoms

Common Symptoms of Pheochromocytoma

  • High blood pressure
  • Headaches
  • Sweating
  • Palpitations
  • Anxiety
  • Nausea and vomiting
  • Weight loss
  • Tremors
  • Fatigue

Less Common Symptoms of Pheochromocytoma

  • Abdominal pain
  • Diarrhea
  • Flushing of the skin
  • Shortness of breath
  • Visual disturbances
  • Frequent urination
  • Chest pain
  • Confusion

If you experience any of these symptoms, it is important to seek medical attention as soon as possible. Early diagnosis and treatment of pheochromocytoma can help prevent serious complications.

Take the Pheochromocytoma Assessment

Causes

Genetic Factors

Pheochromocytoma can be caused by genetic mutations, which can be inherited from a parent or occur spontaneously. The most common genetic mutations associated with pheochromocytoma are in the genes VHL, RET, NF1, and SDHB. People with these mutations have an increased risk of developing pheochromocytoma.

Other Conditions

People with certain other conditions may also be at an increased risk of developing pheochromocytoma. These conditions include multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau syndrome, neurofibromatosis type 1, and familial paraganglioma syndrome.

Age

Pheochromocytoma is more common in adults than in children. The average age of diagnosis is between 40 and 50 years old.

Gender

Pheochromocytoma is more common in women than in men.

Getting a Diagnosis

Medical History and Physical Exam

The first step in diagnosing pheochromocytoma is to take a detailed medical history and perform a physical exam. During the medical history, the doctor will ask about any symptoms that may be related to pheochromocytoma, such as high blood pressure, headaches, palpitations, sweating, and anxiety. The physical exam will look for signs of high blood pressure, such as a rapid pulse, and may also include a check of the abdomen for any lumps or masses.

Imaging Tests

Imaging tests are used to look for tumors in the adrenal glands. The most common imaging tests used to diagnose pheochromocytoma are computed tomography (CT) scans and magnetic resonance imaging (MRI) scans. These tests can help the doctor to determine the size and location of the tumor.

Blood Tests

Blood tests are used to measure levels of hormones and other substances in the blood that can indicate the presence of a pheochromocytoma. These tests can measure levels of catecholamines, which are hormones released by the tumor, as well as other substances such as metanephrines and vanillylmandelic acid.

Urine Tests

Urine tests can also be used to measure levels of catecholamines and other substances in the urine that can indicate the presence of a pheochromocytoma. These tests can measure levels of catecholamines, metanephrines, and vanillylmandelic acid.

Biopsy

If imaging tests and blood and urine tests indicate the presence of a pheochromocytoma, a biopsy may be performed to confirm the diagnosis. During a biopsy, a small sample of tissue is taken from the tumor and examined under a microscope. This can help the doctor to determine if the tumor is cancerous or benign.

Conclusion

Diagnosis of pheochromocytoma is important to ensure proper treatment and management of the condition. Medical history and physical exam, imaging tests, blood tests, urine tests, and biopsy are all used to diagnose pheochromocytoma.

Questions for Your Doctor

Symptoms

Describe any symptoms you have been experiencing, such as headaches, sweating, palpitations, chest pain, and anxiety. Be sure to mention any other symptoms that may be related to pheochromocytoma, such as high blood pressure, weight loss, and fatigue.

Family History

If you have a family history of pheochromocytoma, it is important to tell your doctor. This can help them determine if you are at an increased risk for developing the condition.

Diagnostic Tests

Your doctor may recommend certain tests to diagnose pheochromocytoma, such as a urine test, blood test, or imaging tests. Ask your doctor about the risks and benefits of each test and what the results may mean.

Treatment Options

If you are diagnosed with pheochromocytoma, your doctor will discuss treatment options with you. These may include medications, surgery, or radiation therapy. Ask your doctor about the risks and benefits of each treatment option and how it may affect your quality of life.

Follow-up Care

Your doctor may recommend follow-up care after treatment. Ask your doctor what type of follow-up care is recommended and how often you should have it. This can help ensure that your condition is being monitored and that any changes are addressed quickly.

Treatment

Surgery

Surgery is the primary treatment for pheochromocytoma. The goal of surgery is to remove the tumor and any affected tissue. Depending on the size and location of the tumor, the surgeon may remove the entire adrenal gland or just the tumor itself. In some cases, the tumor may be too large or too close to other organs to be safely removed.

Medication

After surgery, medication may be prescribed to control blood pressure and other symptoms. Alpha-blockers, beta-blockers, and calcium channel blockers are commonly used to control blood pressure. Other medications may be prescribed to control heart rate, reduce anxiety, and reduce the risk of stroke.

Radiation Therapy

In some cases, radiation therapy may be used to treat pheochromocytoma. Radiation therapy uses high-energy beams to destroy cancer cells. It is typically used when the tumor is too large or too close to other organs to be safely removed with surgery.

Chemotherapy

Chemotherapy is a type of cancer treatment that uses drugs to destroy cancer cells. It is typically used when the tumor has spread to other parts of the body or when the tumor is too large to be safely removed with surgery. Chemotherapy may also be used in combination with radiation therapy.

Drugs & Medications

Drugs Used to Treat Pheochromocytoma

The drugs used to treat pheochromocytoma depend on the type and severity of the condition. Commonly used medications include alpha-blockers, beta-blockers, and calcium channel blockers. Alpha-blockers are used to reduce the production of catecholamines, while beta-blockers and calcium channel blockers are used to reduce the effects of catecholamines on the body.

Alpha-Blockers

Alpha-blockers are medications that block the action of the hormone adrenaline. Commonly used alpha-blockers for pheochromocytoma include phenoxybenzamine, prazosin, and doxazosin. These medications can help reduce the production of catecholamines and reduce the symptoms of pheochromocytoma.

Beta-Blockers

Beta-blockers are medications that block the action of the hormone epinephrine. Commonly used beta-blockers for pheochromocytoma include propranolol, atenolol, and metoprolol. These medications can help reduce the effects of catecholamines on the body, such as high blood pressure, headaches, and sweating.

Calcium Channel Blockers

Calcium channel blockers are medications that block the action of calcium in the body. Commonly used calcium channel blockers for pheochromocytoma include verapamil and diltiazem. These medications can help reduce the effects of catecholamines on the body, such as high blood pressure, headaches, and sweating.

Conclusion

Pheochromocytoma is a rare tumor of the adrenal glands that produces hormones called catecholamines. Treatment for pheochromocytoma usually involves surgery to remove the tumor, and medications to control the symptoms. Commonly used medications include alpha-blockers, beta-blockers, and calcium channel blockers. These medications can help reduce the production and effects of catecholamines on the body.

Diet

What to eat

A healthy diet for pheochromocytoma should include plenty of fresh fruits and vegetables, whole grains, lean proteins, and healthy fats. It should also be low in sodium, added sugars, and processed foods.

  • Fruits and vegetables: Aim to eat a variety of fresh fruits and vegetables every day. Choose dark, leafy greens, such as spinach and kale, and brightly colored fruits and vegetables, such as oranges, carrots, and tomatoes.
  • Whole grains: Choose whole grains, such as oats, quinoa, and brown rice, instead of refined grains, such as white bread and white rice.
  • Lean proteins: Choose lean proteins, such as fish, chicken, and beans, instead of red meat.
  • Healthy fats: Choose healthy fats, such as olive oil, nuts, and avocados, instead of unhealthy fats, such as butter and lard.

What to avoid

When living with pheochromocytoma, it is important to avoid certain foods and drinks that can worsen symptoms or increase the risk of complications.

  • Sodium: Avoid processed and packaged foods, which are often high in sodium. Also, limit your intake of salty foods, such as chips, pretzels, and salted nuts.
  • Added sugars: Avoid sugary drinks, such as soda and fruit juice, and processed foods, such as candy and cookies, which are often high in added sugars.
  • Caffeine: Avoid caffeine, which can worsen symptoms and increase blood pressure.
  • Alcohol: Avoid alcohol, which can worsen symptoms and increase the risk of complications.

Conclusion

Eating a healthy diet can help manage symptoms and reduce the risk of complications when living with pheochromocytoma. Aim to eat plenty of fresh fruits and vegetables, whole grains, lean proteins, and healthy fats. Avoid processed and packaged foods, sugary drinks, caffeine, and alcohol.

Lifestyle

Eat a Healthy Diet

Eating a healthy diet is important for everyone, but it is especially important for people with pheochromocytoma. Eating a balanced diet that is low in sodium and high in fiber can help reduce blood pressure and other symptoms. It is also important to avoid processed foods and foods high in sugar, as these can worsen symptoms. Eating smaller meals throughout the day can also help manage symptoms.

Exercise Regularly

Regular exercise can help reduce symptoms of pheochromocytoma, such as high blood pressure and headaches. It is important to talk to your doctor before starting any exercise program, as some exercises may be too strenuous for people with pheochromocytoma. Low-impact exercises such as walking, swimming, and yoga are good options for people with pheochromocytoma.

Manage Stress

Stress can worsen symptoms of pheochromocytoma, so it is important to find ways to manage stress. Relaxation techniques such as deep breathing, meditation, and yoga can help reduce stress. It is also important to get enough sleep and to take time for yourself to relax and unwind.

Monitor Blood Pressure

High blood pressure is a common symptom of pheochromocytoma, so it is important to monitor your blood pressure regularly. Your doctor may recommend that you use a home blood pressure monitor to keep track of your blood pressure. It is also important to take any medications prescribed by your doctor to help control your blood pressure.

Stay Hydrated

Staying hydrated is important for everyone, but it is especially important for people with pheochromocytoma. Drinking plenty of water can help reduce symptoms such as headaches and fatigue. It is also important to avoid caffeine and alcohol, as these can worsen symptoms.

Talk to Your Doctor

It is important to talk to your doctor about any lifestyle changes you are considering. Your doctor can help you develop a plan that is tailored to your individual needs and can help you manage your symptoms. Your doctor can also provide advice on how to best manage your pheochromocytoma.