Diseases and Conditions

Pheochromocytoma

Treatment

The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your doctor will likely prescribe specific blood pressure medications that block the actions of the high-adrenaline hormones to lower the risk of developing dangerously high blood pressure during surgery.

Preoperative preparations

You'll likely take two drugs for seven to 10 days that help lower blood pressure before surgery. These drugs will either replace or be added to other blood pressure drugs you take.

  • Alpha blockers. These drugs keep smaller arteries and veins open and relaxed, improving blood flow and decreasing blood pressure. Alpha blockers include phenoxybenzamine, doxazosin (Cardura) and prazosin (Minipress). Side effects might include irregular heartbeat, dizziness, fatigue, vision problems, sexual dysfunction in men and swelling in the limbs.
  • Beta blockers. These drugs cause your heart to beat more slowly and with less force. They also help keep blood vessels open and relaxed. In your surgery preparation, your doctor will likely prescribe a beta blocker several days after starting the alpha blocker.

    Beta blockers include atenolol (Tenormin), metoprolol (Lopressor, Toprol-XL) and propranolol (Inderal, Innopran XL). Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs.

  • High-salt diet. Alpha and beta blockers widen the blood vessels, causing the amount of fluid within the blood vessels to be low. This can cause dangerous drops in blood pressure with standing. A high-salt diet will draw more fluid inside the blood vessels, preventing the development of low blood pressure during and after surgery.

Surgery

In most cases, your surgeon removes the entire adrenal gland with a pheochromocytoma with minimally invasive surgery. Your surgeon will make a few small openings through which he or she inserts wandlike devices equipped with video cameras and small tools.

The remaining healthy adrenal gland carries out the functions normally performed by two. Blood pressure usually returns to normal.

In some cases, such as when the other adrenal gland has been removed, your doctor might remove only the tumor, sparing some healthy tissue.

If a tumor is cancerous, the tumor and other cancerous tissue will be removed. However, even if all of the cancerous tissue isn't removed, surgery might limit hormone production and provide some blood pressure control.

Cancer treatments

Very few pheochromocytomas are cancerous. As such, research about the best treatments is limited. Treatments for cancerous tumors and cancer that has spread in the body, related to a pheochromocytoma, include:

  • MIBG. This radiation therapy combines MIBG, a compound that attaches to adrenal tumors, with a type of radioactive iodine. The treatment goal is to deliver radiation therapy to a specific site and kill cancerous cells.
  • Peptide receptor radionuclide therapy (PRRT). PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance. It allows radiation to be delivered directly to the cancer cells. One PRRT drug, lutetium Lu 177 dotatate (Lutathera), is used to treat advanced neuroendocrine tumors.
  • Chemotherapy. Chemotherapy is the use of powerful drugs that kill fast-growing cancer cells.
  • Radiation therapy. This may be used for symptomatic treatment of tumors that have spread to the bone, for example, that are causing pain.
  • Targeted cancer therapies. These medications hinder the function of naturally occurring molecules that promote the growth and spread of cancerous cells.