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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. Rarely, this kind of tumor occurs outside the adrenal gland, 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.

    Symptoms

    • Abdominal pain
    • Chest pain
    • Irritability
    • Nervousness
    • Pallor
    • Palpitations
    • Rapid heart rate
    • Severe headache
    • Sweating
    • Weight loss

    Other symptoms that can occur with this disease:

    • Hand tremor
    • High blood pressure
    • Sleeping difficulty

    Symptoms occur in discrete attacks at unpredictable intervals and usually last 15 to 20 minutes. The attacks may increase in frequency, length, and severity as the tumor grows. High blood pressure may occur only from time to time.

    Exams and Tests

    The doctor will perform a physical exam. You may have high blood pressure, rapid heart rate, and fever during an attack of symptoms. Your vital signs can be normal at other times.

    Tests include:

    • Abdominal CT scan
    • Adrenal biopsy
    • Catecholamines blood test
    • Glucose test
    • Metanephrine blood test
    • MIBG scintiscan
    • MRI of abdomen
    • Urine catecholamines
    • Urine metanephrines

    Treatment

    Treatment involves removing the tumor with surgery. It is important to stabilize blood pressure and pulse with medication before surgery. You may need to stay in the hospital with close monitoring of your vital signs.

    After surgery, it is necessary to continually monitor all vital signs in an intensive care unit. When the tumor cannot be surgically removed, medication is needed to manage it. This usually requires a combination of medications to control the effects of the excessive hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.

    Outlook (Prognosis)

    Most patients who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in less than 10% of these patients. Levels of the hormones norepinephrine and epinephrine return to normal after surgery.

    Possible Complications

    High blood pressure may continue in about 1 in 4patients after surgery. However, standard treatments can usually control high blood pressure. In about1 in 10 people, the tumor may return. Patients who have been successfully treated for pheochromocytoma should have testing from time to time to make sure the tumor hasn't returned. Close family members may also benefit from testing, depending on the exact type of tumor.

    When to Contact a Medical Professional

    Call your health care provider if:

    • You have symptoms of pheochromocytoma
    • You had a pheochromocytoma in the past and your symptoms return

    References

    National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine tumors. 2012. Version 1.2012.

    Young WF Jr. Adrenal medulla, catecholamines, and pheochromocytoma. In Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 235.

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      Tests for Pheochromocytoma

        Review Date: 9/9/2012

        Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

        The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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