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Pheochromocytoma

Chromaffin tumors; Paraganglionoma

 

Pheochromocytoma 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.

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. In rare cases, this kind of tumor occurs outside the adrenal gland. When it does, it is usually somewhere else in the abdomen.

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 disorder 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
  • Heart palpitations
  • Sweating
  • High blood pressure

As the tumor grows, the attacks often increase in frequency, length, and severity.

Other symptoms that may occur include:

  • Abdominal or chest pain
  • Irritability, nervousness
  • Pallor
  • Weight loss
  • 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:

  • Abdominal CT scan
  • Adrenal biopsy
  • Catecholamines blood test (serum catecholamines)
  • Glucose test
  • Metanephrine blood test (serum metanephrine)
  • MIBG scintiscan
  • MRI of abdomen
  • Urine catecholamines
  • Urine metanephrines
  • 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 too much 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 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

 

Call 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

Cusack JC, Phitayakorn R. The management of pheochromocytoma. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 11th ed. Philadelphia, PA: Elsevier Saunders; 2014:636-642.

National Cancer Institute. Pheochromocytoma and paraganglioma treatment (PDQ) - health professional version. Updated July 10, 2015. Cancer.gov. www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_38_toc. Accessed October 22, 2016.

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.

 
  • Endocrine glands

    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 pace of chemical activity 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

    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

    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

    Adrenal gland hormone secretion - illustration

    Adrenal glands produce hormones such as estrogen, progesterone, steroids, cortisol, and cortisone, and chemicals such as adrenalin (epinephrine), norepinephrine, and dopamine. When the glands produce more or less hormones than required by the body, disease conditions may occur.

    Adrenal gland hormone secretion

    illustration

    • Endocrine glands

      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 pace of chemical activity 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

      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

      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

      Adrenal gland hormone secretion - illustration

      Adrenal glands produce hormones such as estrogen, progesterone, steroids, cortisol, and cortisone, and chemicals such as adrenalin (epinephrine), norepinephrine, and dopamine. When the glands produce more or less hormones than required by the body, disease conditions may occur.

      Adrenal gland hormone secretion

      illustration

    Tests for Pheochromocytoma

     

       

      Review Date: 8/15/2016

      Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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