Definition

Pheochromocytoma is a tumor. It is made up of special adrenal gland cells. The cells secrete hormones such as epinephrine and norepinephrine. These hormones help to regulate the heart rate and blood pressure. The cells may secrete excessive amounts of the hormones. This results in periods of:

    
  • Very high blood pressure
  • Rapid heart beat or palpitations
  • Excessive sweating
  • Severe headaches

    • Most of these tumors are benign and grow on the adrenal glands, which are located on top of the kidneys. Other tumors occur elsewhere in the body.

    Adrenal Glands

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    Causes

    In most cases, pheochromocytoma has no known cause. In others, it is caused by a genetic mutation.

    Risk Factors

    Factors that may increase your chances of pheochromocytoma include:

        
  • A family history
  • Tumors in other glands of the body
  • Hormonal disorders

    • Genetic diseases associated with pheochromocytoma include:

        
  • Von Hippel-Lindau disease
  • Multiple endocrine neoplasia, type 2
  • Neurofibromatosis type 1
  • Paraganglioma syndromes

    • Symptoms

    Most people don't have symptoms. In people who have them, symptoms may include:

        
  • Severe headaches
  • Excessive sweating
  • Fast heart rate
  • Sensation of a panic attack
  • Blurred vision
  • Nausea, vomiting, constipation
  • Pounding heart beat
  • Chest pain
  • Involuntary trembling
  • Pain in the lower chest or upper abdomen
  • Warmth, flushing
  • Increased appetite
  • Weight loss
  • Insomnia
  • High blood pressure, which can be constant or sporadic
  • Tingling, burning, or numbness in the legs and feet
  • Shortness of breath
  • Muscle weakness
  • Anxiety
  • Unable to cope with high temperature
  • Aggressive or unusual behavior

    • Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will be done.

    Your doctor may need to test your body fluids. This can be done with:

        
  • Urine testing
  • Blood testing
  • Clonidine suppression test

    • Your doctor may need images of your body structures. This can be done with:

        
  • CT scan
  • Ultrasound
  • MRI
  • MIBG scintiscan, or adrenal medullary imaging
  • PET scan

    • Treatment

    Laparoscopic Adrenalectomy

    If the tumor is benign, it is removed. This procedure uses small incisions and special instruments. Sometimes, the adrenal glands are removed as a part of this process.

    Medications

    Prior to surgery, high blood pressure will need to be brought under control using blood pressure lowering medications.

    Chemotherapy and Radiation Therapy

    Chemotherapy may be used if the tumor is cancerous and has spread outside of the adrenal glands.

    Radiation therapy may also be used to help destroy the tumors if they have spread.

    Prevention

    There are no current guidelines to prevent pheochromocytoma.