How Pheochromocytoma Is Treated

Pheochromocytoma is a rare tumor that can develop in the adrenal glands. These growths are most often benign (noncancerous), but, in some cases, they can be malignant (cancerous).

Pheochromocytomas release excess amounts of fight-or-flight hormones called catecholamines, which can produce symptoms such as high blood pressure, heart palpitations, heavy sweating, and an impending sense of doom.

Symptoms tend to occur in episodes, known as paroxysms, that can be life-threatening in rare cases. If you have symptoms, it's important to see your healthcare provider for a prompt diagnosis and treatment, if needed.

If you are diagnosed with a pheochromocytoma, surgery to remove the tumor is the preferred treatment. Other treatment options may include chemotherapy, radiation, and immunotherapy if the tumor has spread.

This article will review the treatment options for pheochromocytoma.

Close up of laporascopic surgery

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Lifestyle/Home Remedies

The symptoms from pheochromocytomas are episodic, meaning they come and go without warning. You may experience a rise in blood pressure, increased sweating, or your heart rate may go up. Avoiding stressful scenarios or triggers for these events may help manage symptoms.

There are no current supplements or alternative lifestyle approaches that have been adopted for the treatment of pheochromocytoma.

Medications

If you are diagnosed with a pheochromocytoma, your healthcare provider will likely prescribe medication to help counteract the effects of the excess catecholamines in your system and control your blood pressure.

Usually, one to three weeks before having surgery to remove a pheochromocytoma, your healthcare provider will start you on a blood pressure medication called an alpha-blocker. The most common drug that is used is Dibenzylene (phenoxybenzamine).

This protocol is needed to reduce the release of catecholamines, as these could cause a life-threatening high blood pressure crisis during surgery.

Surgeries and Procedures

The most successful treatment for pheochromocytoma is the surgical removal of the tumor from the adrenal gland. This procedure is called an adrenalectomy. In many cases, surgery is considered curative.

Laparoscopic surgery is the preferred method for removal. This minimally invasive procedure is done using a thin, camera-equipped surgical tool that is inserted through a small incision in the abdomen.

If tumors are present in both adrenal glands, management must be individualized. Many surgeons recommend trying to save part of an adrenal gland as it is very uncommon for these tumors to be cancerous.

If both adrenal glands are removed, lifelong therapy is required to replace the hormones lost. Even with replacement, people may still develop acute adrenal crisis (Addison's disease or Addisonian crisis), a condition in which the body has insufficient levels of cortisol and other adrenal hormones to manage critical functions.

Chemotherapy

For tumors that are growing rapidly or have spread, chemotherapy may be recommended. This is a medication that kills cancer cells. It can be given orally by pill or by intravenous (IV) infusion.

Pheochromocytomas are usually treated using a combination of several drugs.

Radiation

A radiopharmaceutical is often recommended for people with metastatic tumors, or those who aren't eligible for surgery.

In this therapy, a specialized radioactive substance is injected into a vein. Then this substance accumulates in pheochromocytoma tumor cells. As it is taken up by the tumor cells, it gives off radiation that results in the tumor cells' death.

Alternative Treatments

Targeted therapies: These are drugs that specifically target cancer cells or a pathway involved in their growth. Oncologists (doctors who specialize in cancer treatment) have had some success using the targeted therapy drug Sutent (sunitinib) in people with pheochromocytoma. The target therapy drug Welireg (belzutifan) was approved by the Food and Drug Administration (FDA) in 2021 for advanced metastatic pheochromocytoma.

Immunotherapy: Immunotherapy drugs are medications that use the immune system or principles of the immune system to treat tumors and have been successful with some other types of tumors. Their use is just beginning to be explored with pheochromocytoma, but at least one study has found that an immunotherapy drug (in combination with a targeted therapy) worked to slow or step progression of metastatic pheochromocytoma, helping to extend patients' survival.

Follow-Up Treatment and Screening

After surgery for early-stage tumors, blood work is usually checked a few weeks after the procedure. If normal, further treatment may not be needed at the time. Fortunately, a significant number of people will have their blood pressure return to normal after the tumor is removed.

Lifelong monitoring usually is required after treatment to determine if the tumor will recur, to know whether the tumor is benign or malignant (which sometimes takes months after surgery to determine), and to monitor for recurrence.

If you had both adrenal glands removed, you will require lifelong replacement of adrenal hormones. If you only had one adrenal gland removed, your remaining adrenal gland will compensate and take the place of both.

In addition, since many of these tumors occur as part of a genetic syndrome, long-term screening is needed to manage other manifestations of the syndrome. If genetic testing for associated genetic syndromes and gene mutations was not done previously, this will also be done during follow-up.

Prognosis

The survival rate for pheochromocytoma operations is very high. Estimates show that nearly 95% of patients are alive after five years.

For people who have localized tumors, between 7% and 17% will develop a recurrence. The five-year survival rate for metastatic pheochromocytoma or a local/regional pheochromocytoma that later recurs is 40%–45%.

Summary

Surgery is the preferred treatment for pheochromocytoma. This minimally invasive procedure is called adrenalectomy and, in many cases, is curative. If the tumor has spread or is inoperable, additional cancer-killing medications may be used, including radioactive pharmaceuticals, chemotherapy, targeted therapy drugs, and immunotherapy.

A Word From Verywell

Going through treatment for a pheochromocytoma is stressful and scary, so it's important to reach out to friends and loved ones for additional support. They can help you arrange meals, coordinate rides to and from therapy, and offer a shoulder to cry on. There may also be support groups either online or locally in which you can connect with people who have gone through or are going through a similar experience.

Fortunately, the outlook after treatment for most pheochromocytomas is positive. If you can advocate for yourself and stay strong through treatment, you'll be able to get back on track to living a long, happy, and fulfilling life.

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4 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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