Pheochromocytoma
Chromaffin tumors; ParaganglionomaPheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure.
Tumor
A tumor is an abnormal growth of body tissue. Tumors can be cancerous (malignant) or noncancerous (benign).
Causes
Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney. In rare cases, a pheochromocytoma occurs outside the adrenal gland. When it does, it is usually somewhere else in the abdomen.
Adrenal glands
The adrenal glands are two small triangle-shaped glands in the upper abdomen. One gland is located on top of each kidney.
Very few pheochromocytomas are cancerous.
The tumors may occur at any age, but they are most common from early to mid-adulthood.
In few instances, the condition may also be seen among family members (hereditary).
Symptoms
Most people with this tumor have attacks of a set of symptoms, which happen when the tumor releases hormones. The attacks usually last from a few minutes to hours. The set of symptoms include:
- Headaches
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Heart palpitations
Heart palpitations
Palpitations are feelings or sensations that your heart is pounding or racing. They can be felt in your chest, throat, or neck. You may:Have an unpl...
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- High blood pressure
As the tumor grows, the attacks often increase in frequency, length, and severity.
Other symptoms that may occur include:
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Abdominal or chest pain
Abdominal
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
ImageRead Article Now Book Mark Article - Irritability, nervousness
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Pallor
Pallor
Paleness is an abnormal loss of color from normal skin or mucous membranes.
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- Nausea and vomiting
- Shortness of breath
- Seizures
- Problems sleeping
Exams and Tests
The health care provider will perform a physical exam. You'll be asked about your medical history and symptoms.
Tests done may include:
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Abdominal CT scan
Abdominal CT scan
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
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Adrenal biopsy
Adrenal biopsy
A biopsy is the removal of a small piece of tissue for laboratory examination.
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Catecholamines blood test (serum catecholamines)
Catecholamines blood test
This test measures the levels of catecholamines in the blood. Catecholamines are hormones made by the adrenal glands. The three catecholamines are ...
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Glucose test
Glucose test
A blood sugar test measures the amount of a sugar called glucose in a sample of your blood. Glucose is a major source of energy for most cells of the...
ImageRead Article Now Book Mark Article - Metanephrine blood test (serum metanephrine)
- An imaging test called an MIBG scintiscan
MIBG scintiscan
An MIBG scintiscan is a type of imaging test. It uses a radioactive substance (called a tracer). A scanner finds or confirms the presence of pheoch...
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MRI of abdomen
MRI of abdomen
An abdominal magnetic resonance imaging scan is an imaging test that uses powerful magnets and radio waves. The waves create pictures of the inside ...
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Urine catecholamines
Urine catecholamines
Catecholamines are chemicals made by nerve tissue (including the brain) and the adrenal gland. The main types of catecholamines are dopamine, norepin...
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- PET scan of abdomen
Treatment
Treatment involves removing the tumor with surgery. It is important to stabilize your blood pressure and pulse with certain medicines before surgery. You may need to stay in the hospital and have your vital signs closely monitored around the time of surgery. After surgery, your vital signs will be continuously monitored in an intensive care unit.
When the tumor cannot be surgically removed, you will need to take medicine to manage it. A combination of medicines is usually needed to control the effects of the extra hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.
Outlook (Prognosis)
Most people who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in some people. Levels of the hormones norepinephrine and epinephrine return to normal after surgery.
Continued high blood pressure may occur after surgery. Standard treatments can usually control the high blood pressure.
People who have been successfully treated for pheochromocytoma should have testing from time to time to make sure the tumor has not returned. Close family members may also benefit from testing, because some cases are inherited.
When to Contact a Medical Professional
Contact your provider if you:
- Have symptoms of pheochromocytoma, such as headache, sweating, and palpitations
- Had a pheochromocytoma in the past and your symptoms return
References
Brigode WM, Miraflor EJ, Palmer BJA. The management of pheochromocytoma. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 13th ed. Philadelphia, PA: Elsevier; 2020:750-756.
National Cancer Institute website. Pheochromocytoma and paraganglioma treatment (PDQ) - health professional version. Cancer.gov. www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_38_toc. Updated August 25, 2022. Accessed October 13, 2022.
Pacak K, Timmers HJLM, Eisenhofer G. Pheochromocytoma. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric.7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 110.
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Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
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Adrenal metastases - CT scan - illustration
This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.
Adrenal metastases - CT scan
illustration
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Adrenal Tumor - CT - illustration
CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.
Adrenal Tumor - CT
illustration
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Adrenal gland hormone secretion - illustration
The adrenal gland secretes steroid hormones such as cortisol and aldosterone. It also makes precursors that can be converted to sex steroids (androgen, estrogen). A different part of the adrenal gland makes adrenaline (epinephrine). When the glands produce more or less hormones than required by the body, disease conditions may occur.
Adrenal gland hormone secretion
illustration
-
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
-
Adrenal metastases - CT scan - illustration
This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.
Adrenal metastases - CT scan
illustration
-
Adrenal Tumor - CT - illustration
CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.
Adrenal Tumor - CT
illustration
-
Adrenal gland hormone secretion - illustration
The adrenal gland secretes steroid hormones such as cortisol and aldosterone. It also makes precursors that can be converted to sex steroids (androgen, estrogen). A different part of the adrenal gland makes adrenaline (epinephrine). When the glands produce more or less hormones than required by the body, disease conditions may occur.
Adrenal gland hormone secretion
illustration
Review Date: 8/15/2022
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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