Pheochromocytoma is a rare type of tumor that usually grows in the adrenal glands, above the kidneys. It is also known as an adrenal paraganglioma or a chromaffin cell tumor. It is generally common in adults between the ages of 30 to 50, though people of all ages might have it. Ten percent of all cases are known to occur among children.
Your adrenal glands produce hormones for controlling things like your metabolism and your blood pressure. A pheochromocytoma releases hormones as well, at much higher levels than normal. These extra hormones can lead to high blood pressure, and this might lead to damage to several of your organs, such as your brain, heart, lungs, and kidneys.
Though most pheochromocytoma tumors are benign, around 10 to 15 percent of them are generally cancerous, and they might spread to other parts of the body.
People having these tumors suffer from high blood pressure all the time. For others, it can go up and down.
Most people also have at least one or more of the following symptoms:
- Dizziness while standing
- Pale skin
- Fast or uneven heartbeat
- Severe headache
- Shortness of breath
- Unusual sweating
- Stomach, side, or back pain
- Anxiety attacks
- Unintended weight loss
These symptoms can occur suddenly, like an attack, multiple times during a day. For some people, they might occur only a few times a month. As the tumor grows, the attacks can get stronger and might occur more often.
In some people, the attacks might be triggered by certain things such as:
- Pressure on the tumor
- Certain medications
- Physical activity
- Too much emotional stress
- Foods containing excessive amino acid tyramine, such as red wine, chocolate, or cheese
Causes & risk factors
Researchers are still unsure of what exactly is the cause of pheochromocytoma. This tumor develops in specialized cells, which are termed chromaffin cells, located in the center of an adrenal gland.
These cells release certain hormones, primarily adrenaline, and noradrenaline that helps to control several body functions, such as heart rate, blood pressure as well as blood sugar.
Adrenaline and noradrenaline trigger your body’s fight-or-flight response when a threat is perceived. The hormones can cause your blood pressure to increase as well as your heart to beat faster. They also prepare other body systems in order to help enable you to react quicker. A pheochromocytoma causes more of these hormones to be released even when you’re not in a threatening situation.
People having certain rare inherited disorders are at an increased risk of pheochromocytoma or paraganglioma. It is also likely that tumors associated with these disorders will be cancerous.
These genetic conditions include the following:
Multiple endocrine neoplasias, type 2 (MEN 2) – This disorder results in tumors in more than a single part of the body’s hormone-producing (endocrine) system. Other tumors which are associated with this condition may appear on the thyroid, tongue, parathyroid, lips, as well as the gastrointestinal tract.
Neurofibromatosis 1 (NF1) – This can result in multiple tumors in the skin, pigmented skin spots as well as tumors of the optic nerve.
Von Hippel-Lindau disease – This disorder can result in tumors at multiple sites, which can include the central nervous system, endocrine system, pancreas as well as kidneys.
Hereditary paraganglioma syndromes – These are inherited disorders which may result in paragangliomas or pheochromocytomas.
Your doctor might require multiple tests for diagnosing this condition. These can include the following:
- PET imaging
- Blood plasma test for catecholamine and metanephrine levels
- Laboratory tests in order to assess the hormone levels
- Urine metanephrines test for catecholamine and metanephrine levels
Generally, the primary treatment for pheochromocytoma is surgery for removing the tumor. However, before you go for surgery, your doctor should likely recommend certain blood pressure medications. These are meant to block the actions of the high-adrenaline hormones and to lower the risk of developing extreme high blood pressure.
You will likely receive few drugs which can include alpha-blockers, for improving blood flow and decreasing blood pressure and beta-blockers, to make your heart beat slowly with less force.
It is also likely that your healthcare team will instruct you to consume a high-salt diet. These medications can cause a huge drop in your blood pressure; a high-salt diet will help you draw more fluid inside the blood vessels, and prevent you from having low blood pressure during and after the surgery.
During the surgery, in most cases, the surgeon removes the entire adrenal gland with a pheochromocytoma using minimally invasive surgery. For this, your surgeon will need to create a few tiny openings through which he/she will insert wandlike devices equipped with video cameras and small tools.
The remaining healthy adrenal gland is able to carry out the functions normally performed by two. Blood pressure will likely return to normal within a short time.
If the other adrenal gland has been removed, then during the surgery, your doctor will remove only the tumor, and spare some of the healthy tissue.
If a tumor is cancerous, the tumor and other cancerous tissues are going to be removed. However, even if all of the cancerous tissues are not removed, surgery might limit the production of hormones. It also helps in providing some control over the blood pressure.
Cancerous pheochromocytoma treatments
Certains pheochromocytomas are also cancerous. And therefore, other treatments can be required for them, such as:
This is a special kind of radiation therapy. It combines MIBG, a compound that attaches to the adrenal tumors, with a type of radioactive iodine. The goal of the treatment is to deliver radiation therapy to a specific site and eliminate the cancerous cells.
This treatment might be used for symptomatic treatment of tumors that have spread to other parts of the body and causing pain.
Targeted cancer therapies
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 to the cancer cells directly.
People having pheochromocytoma which is not cancerous generally have a 5-year survival rate of 96 percent, while for people with a cancerous tumor, the survival rate is 44 percent.
For ensuring successful treatment, an early diagnosis is not always enough. It is best that you seek out a surgeon who is highly skilled and experienced.
High blood pressure caused by the tumors can cause damage to various organs, especially tissues of the cardiovascular system, brain, or even the kidneys. Some of the critical conditions include the following:
- Heart disease
- Kidney failure
- Problems with the nerves of the eye