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Pheochromocytoma
Pheochromocytoma is a rare tumor that forms in cells that produce and release hormones (neuroendocrine cells). These tumors are found in the center of the adrenal glands, which sit on top of the kidneys.
A pheochromocytoma creates excess hormones that can lead to symptoms such as high blood pressure and a fast heart rate.
Most pheochromocytomas are not cancerous (are benign). Some are cancerous (are malignant) and can spread to other parts of the body (metastasize).
4 Interesting Facts of Pheochromocytoma
- Catecholamine-secreting tumor of chromaffin cells, usually involving adrenal glands
- As with carcinoid, may present with cutaneous flushing and palpitations
- Unlike carcinoid, episodes are also associated with headache, sweating, anxiety, and hypertension
- Differentiated on basis of history, physical examination, elevated metanephrine and catecholamine levels in serum and urine, and presence of adrenal tumor on CT or MRI
- Similar presentation to opioid withdrawal: anxiety, diaphoresis, and tachycardia; hypertension and palpitations are also common
- Differentiated by elevated metanephrine and catecholamine levels in serum and urine, and presence of adrenal tumor on CT or MRI. Also, urine drug screen result is usually negative for opioids
What are the causes?
The cause of this condition is not known.
What increases the risk?
The following factors may make you more likely to develop this condition:
- Being 40–60 years of age.
- Being male.
- Having certain inherited (genetic) syndromes, such as multiple endocrine neoplasia type 1 or 2.
- Having a weakened immune system due to:
- Medicines (immunosuppressants).
- Infection with HIV (human immunodeficiency virus).
What are the symptoms?
Symptoms may be triggered by certain medicines and foods. Other factors that can trigger symptoms (trigger a spell) may include physical exertion, anxiety, stress, anesthesia, and changes in body position. Symptoms can also occur without a trigger.
Symptoms of this condition include:
- High blood pressure.
- Shortness of breath.
- Headaches.
- Fever.
- Nausea or vomiting.
- Anxiety attacks.
- A fast or irregular heartbeat (palpitations).
- Pale or clammy skin.
- Heavy sweating.
In some cases, there are no symptoms.
How is this diagnosed?
This condition may be diagnosed based on:
- A physical exam and your medical history.
- Urine tests.
- Blood tests.
- Imaging tests, such as:
- CT scan.
- MRI.
- X-ray.
- PET scan.
- M-iodobenzylguanidine (MIBG) or octreotide scan. These tests use radioactive substances to detect certain tumors.
If you are diagnosed with pheochromocytoma, you may have other tests to see if you have a genetic syndrome that can raise your risk of developing diseases such as cancer. If the tumor is cancerous, diagnosis will also include determining whether the cancer has spread to other parts of the body (what stage it is).
How is this treated?
Treatment for this condition depends on whether the tumor is cancerous and the stage of the cancer. Treatment can include:
- Using medicines to lower blood pressure prior to surgery.
- Having surgery to remove the tumor and surrounding tissue.
- Getting a type of radioactive iodine inserted into the body through an IV tube (radioactive iodine treatment). This substance will find cancer cells and kill them by giving off radiation.
- Using high-energy X-rays or radiation to kill cancer cells or to slow their growth (radiation therapy).
- Using medicines:
- To kill cancer cells or to slow their growth (chemotherapy).
- To attack a tumor’s genes and proteins (targeted therapy). These medicines attack the genes and proteins that allow a tumor to grow while limiting damage to healthy cells.
- Blocking the flow of blood to the tumor (embolization therapy). This helps to kill cancer cells.
- Using radio waves to destroy cancer cells (radiofrequency ablation).
- Freezing tissue to destroy cancer cells (cryoablation).
Follow these instructions at home:
- Learn about your disease and possible side effects from treatment. This will help you talk about your choices with your health care provider and decide on treatments that are right for you.
- Take over-the-counter and prescription medicines only as told by your health care provider.
- Return to your normal activities as told by your health care provider. Ask your health care provider what activities are safe for you.
- Eat a healthy diet. A healthy diet includes lots of fruits and
vegetables, low-fat dairy products, lean meats, and fiber.
- Make sure half your plate is filled with fruits or vegetables.
- Choose high-fiber foods such as whole-grain breads and cereals.
- Limit alcohol intake to no more than 1 drink a day for nonpregnant women and 2 drinks a day for men. One drink equals 12 oz of beer, 5 oz of wine, or 1½ oz of hard liquor.
- Get regular exercise. Aim for 30 minutes of moderate-intensity activity 5 times a week. Examples of moderate-intensity activity include walking and yoga. Be sure to talk with your health care provider before starting any exercise routine.
- Keep all follow-up visits as told by your health care provider. This is important.
Where to Seek Additional Information
- National Cancer Institute: www.cancer.gov
- American Cancer Society: www.cancer.org
- American Society of Clinical Oncology: www.cancer.net
Contact a health care provider if:
- You have abdominal pain or nausea.
- You have diarrhea or a change in bowel movements.
- You have swelling or redness anywhere, especially around a cut or wound.
- You have a headache or sinus pain.
- You have skin sores or a rash.
- You have pain or burning when urinating.
- You have unexplained weight loss.
Get help right away if:
- You have chest pain or shortness of breath.
- You have a severe headache.
- You have a fever.
- You have blood in your urine.
- You are confused.
- You have swelling, pain, or tenderness in your arms or legs.
- You have uncontrolled vomiting.
Summary
- A pheochromocytoma is a tumor that forms in cells that produce and release hormones (neuroendocrine cells). These tumors are found in the center of the adrenal glands, which sit on top of the kidneys.
- A pheochromocytoma creates excess hormones that can lead to symptoms such as high blood pressure and a fast heart rate.
- You are more likely to develop this disease if you have certain inherited (genetic) syndromes.
- Treatment for pheochromocytoma depends on whether the tumor is cancerous and whether it has spread to other parts of the body.
