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Pheochromocytoma is a rare tumor that grows in the medulla region of the adrenal gland. It begins as a non-cancerous (benign) region and later develops into malignancy. The adrenal medulla helps in producing hormones called epinephrine or adrenaline hormone and norepinephrine or noradrenaline, which help during stress or danger. Read More

Top Doctors For Pheochromocytoma Treatments

Top Hospitals For Pheochromocytoma Treatments


What is pheochromocytoma?

Pheochromocytoma is a rare tumor that grows in the medulla region of the adrenal gland. It begins as a non-cancerous (benign) region and later develops into malignancy. The adrenal medulla helps in producing hormones called epinephrine or adrenaline hormone and norepinephrine or noradrenaline, which help during stress or danger. They also help increase blood flow, heart rate, etc. These hormones are produced in excess in pheochromocytomas, affecting the body’s normal physiological responses. Pheochromocytomas can also be found in the abdomen, pelvis, chest, and neck. Recent studies have shown that it occurs in an estimated 1-2 individuals per 100,000 persons per year. Men and women can be affected, especially those in their 40s and 50s; sometimes, it affects people in their 20s. People diagnosed with this condition have high blood pressure as one of its primary symptoms. About 20% to 35% of pheochromocytomas are discovered to be inherited. 

Picture Courtesy: clevelandclinic


Pheochromocytoma can be caused by tumors occurring in the specialized cells that are responsible for the hormone precursor amino acid, which is predominantly found in the adrenal medulla.

What are the risk factors associated with pheochromocytomas?

The risk factors involved include some genetic conditions such as: 


symptoms of pheochromocytoma
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The symptoms of pheochromocytoma include:

  • High blood pressure
  • Severe headaches
  • Increase heart rate
  • Profuse sweating
  • Feeling of hotness
  • Chest pain
  • Anxiety
  • Sometimes, pains in the upper part of the stomach.
  • Constipation may also occur.
  • Irregular heartbeat.

How is pheochromocytoma diagnosed?

diagnosing pheochromocytomas
Picture Courtesy: sciencedirect

One of the major ways of diagnosing pheochromocytomas is by observing the level of the hormones, i.e., epinephrine and norepinephrine, in the body. This can be done by

  • Urine Test; it involves using 24-hour urine collection by a special urine container that has a small amount of preservative. Although it could be inconvenient, it is quite reliable.
  • Blood tests, such as blood plasma tests, can be carried out.
  • Imaging Tests; are done to locate the pheochromocytomas. They include cross-section scans, computed tomography (CT) scans, and magnetic resonance imaging (MRI).
Imaging tests for pheochromocytomas

Axial, T2-weighted magnetic resonance imaging (MRI) scan showing a large left suprarenal mass of high signal intensity on a T2-weighted image. The mass is a pheochromocytoma.

Abdominal computed tomography (CT)
Picture Courtesy: emedicine.medscape

An abdominal computed tomography (CT) scan showing left suprarenal soft-tissue attenuation, which is the paraganglioma.

  • MIBG scan (M-iodobenzylguanidine); It is done to detect pheochromocytomas or paragangliomas.
  • PET scan (positron emission tomography); it is done to locate the location of the tumor anywhere else in the body.
  • Genetic Testing can also be done.

How is pheochromocytoma treated?

treating pheochromocytomas
Picture Courtesy: emedicine.medscape

Adrenalectomy specimen containing pheochromocytoma. A non-neoplastic cortex of the adrenal (yellow) covers a tiny tan-red tumor in the medullary area, which is a pheochromocytoma.

One of the major ways of treating this condition is through surgeries. These surgeries can be performed either through keyhole surgery (laparoscopic adrenalectomy) or open surgery. During this surgery, the whole of the affected adrenal gland is removed by the surgeon. However, before surgery, some medications can be taken that help to block the actions of high adrenaline hormones to reduce the risk of high blood pressure during surgery. Such medications include alpha-blockers such as prazosin, phenoxybenzamine, etc., and beta-blockers, which reduce heart rate, like metoprolol, propranolol, etc. Also, the patient will be asked to increase their salt intake, which aids in balancing the effect of the medications on the blood vessels. Other treatment methods include:

  1. Radiotherapy– There are two types of radiation therapy. They are External radiation therapy- During this therapy, they use a machine outside the body to send radiation toward the cancer.
  2. Internal radiation therapy – During this therapy, they make use of a radioactive substance sealed in needles, seeds, wires or catheters, which the doctor places directly into or near the tumor.
  • Chemotherapy
  • Ablation- They include radiofrequency ablation and cryoablation.
  • Embolization therapy- During this treatment, they block the blood flow to the artery that supplies the adrenal gland, which helps kill the cancer cells that are growing there.
  • Targeted therapy

What are the complications associated with pheochromocytomas?

Since the hormone epinephrine and norepinephrine are major regulators of blood pressure in the body, untreated pheochromocytomas may lead to:

  • Damage to body organs
  • Heart attack, cardiomyopathy (damage to the muscles of the heart)
  • Stroke
  • Kidney failure
  • Pulmonary edema (fluid build-up in the lungs) 
  • Brain hemorrhage (bleeding in the brain)
  • Cancerous cells may travel to the lymph system, liver, sometimes lungs and bones


Are pheochromocytomas dangerous?

Since pheochromocytomas affect one of the essential hormones in the body, the catecholamines, they can be considered dangerous. Catecholamines – epinephrine and norepinephrine, control the regulation of blood flow in the body. They are considered high-risk tumors.

How often are pheochromocytomas cancerous?

Most pheochromocytomas are benign. Only About 10% of them have been recorded to be malignant, which could be due to mutations in the genes.

What food trigger pheochromocytoma?

Foods that are rich in tyramine should be avoided as it tends to increase the rate of blood flow in the body. Such food rich in tyramine includes overripe food, fermented food, cheese, etc.

Is pheochromocytoma dangerous during pregnancy?

Definitely, pheochromocytoma during pregnancy is dangerous to both mother and child. It increases the blood pressure of the mother and can cause damage to the kidney, also affecting the baby’s oxygen and causing premature birth. Generally, it could complicate the pregnancy and delivery.

What is the best test for pheochromocytoma?

A blood plasma test is considered the best for diagnosing pheochromocytomas.

What is the prognosis of pheochromocytoma?

If treated early, patients diagnosed with pheochromocytoma have a good prognosis. In caseIn case the doctor removes both of the adrenal glands, then the patient will be asked to take medications to help replace the hormones that the body won’t be able to make anymore for the rest of their lives. The patient is advised to have regular follow-ups with the doctor to check the patient’s overall health condition. According to the studies, it is estimated that 95% of the patients diagnosed with a cancerous form of pheochromocytoma that hasn’t spread to other parts of the body live for at least 5 years, whereas tumor (cancer) which has spread to other parts of the body or came back after treatment, about 34 to 60% of people live at least 5years after the diagnosis [1]. 

What is the difference between pheochromocytoma and paragangliomas?

Pheochromocytoma and paraganglioma are considered to be both rare forms of tumors that come from the same type of cells called chromaffin cells. Pheochromocytoma is a tumor that forms in the center of the adrenal gland (adrenal medulla), whereas paragangliomas form outside the adrenal gland [2].