These growths produce excess fight-or-flight hormones calledcatecholamines.
Pheochromocytomas are very rare, with only two to eight cases diagnosed per 1 million people each year.
Fewer than 10% of pheochromocytomas aremalignant(cancerous).
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When they form outside the adrenal glands, these tumors are called extra-adrenal paragangliomas or paragangliomas.
In contrast to pheochromocytomas, paragangliomas are less likely to secrete large amounts of catecholamines.
They also have a higher chance of being malignant than pheochromocytomas.
Other symptoms include headaches, heart palpitations, and sweating.
Some of these tumors are also more likely to be malignant than tumors that occur sporadically.
Gene mutations may account for up to 40% of pheochromocytomas.
Otherwise, no environmental, lifestyle, or dietary risk factors have been identified.
Taking a family history is also very important.
Your healthcare provider will also likely order lab tests and imaging scans.
This test is less sensitive, but also less likely to give false-positive readings.
Urine is collected each time a person urinates for 24 hours and then evaluated in the lab.
CT is considered the best test to locate a pheochromocytoma.
Scintigraphy(MIBG scan):Most of the time a pheochromocytoma can be located with CT or MRI.
If a tumor is not seen but strongly suspected, this nuclear imaging study may be done.
Imaging tests, including MRI, CT scan, and scintigraphy (MIBG scan) may also be used.
What Are the Treatment Options?
Treatment may also include chemotherapy, radiation therapy, or other therapies if the tumor has spread.
However, surgery should not be performed immediately.
The most common drug that is used is Dibenzylene (phenoxybenzamine).
Laparoscopic surgeryis the preferred method for removal.
If tumors are present in both adrenal glands, management must be individualized.
If both adrenal glands are removed, lifelong therapy is required to replace the hormones lost.
Additional Treatment Options
Radiopharmaceuticals: A radiopharmaceutical is often recommended first for people with metastatic tumors.
In this therapy, a radioactive substance that accumulates in pheochromocytoma tumor cells is injected into a vein.
Targeted therapies: Targeted therapies are drugs that specifically target cancer cells or a pathway involved in their growth.
It has been successful with some other types of tumors.
Surgery is the preferred treatment option for tumors that have not spread.
It is often curative.
For those who have benign tumors that do not recur, life expectancy is similar to the general population.
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%.
Fortunately, the Internet now allows people to interact with others facing a similar diagnosis worldwide.
High blood pressure is a common symptom of this tumor.
Diagnosing a pheochromocytoma usually involves a physical exam, blood work, urine analysis and imaging tests.
With any medical condition,being your own advocateis a must in today’s medical climate.
Having an understanding of symptoms of pheochromocytoma may aid in your diagnosis.
Knowing about the condition will also help you ask the right questions to get the care you deserve.
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