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We normally have two small adrenal glands that sit atop our kidneys. The center of these glands, known as the medulla, produces hormones that prompt a “fight or flight response” to help our bodies prepare for potentially stressful or dangerous situations. They increase our heart rate, blood pressure and the amount of oxygen we breathe in, as well as temporarily improve eyesight and cause the liver to release extra glucose, or sugar, for immediate energy. It’s what we call an adrenaline rush.
The outer layer of the glands, known as the cortex, produces hormones that control functions of the heart, kidneys, gastrointestinal tract, bones, reproductive organs and our immune system.
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Adrenal tumors can cause either the over-production or under-production of vital hormones. Tumors that cause an over-production of hormones are called functioning tumors. Non-functioning tumors do not produce hormones. Most adrenal tumors are not inherited, but some adrenaline producing tumors and some tumors of the cortex may be the result of a genetic mutation carried through the family. A blood test can determine if genetics plays a part.
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Adrenal cancer is rare (1 in a million). Most adrenal tumors are not cancerous, and may not even require any treatment. However, the long-term effects of over-production of some adrenal gland hormones can lead to severe conditions, such as heart attack and stroke, so diagnosis is important.
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The most common type of adrenal tumors, called adenomas, are non-functioning and non-cancerous tumors of the adrenal cortex. Although non-cancerous, they can over-produce hormones and lead to high blood pressure, low potassium levels, headaches, sweating, palpitations and abdominal obesity (Cushing’s Syndrome). Therefore, treatment ranging from pharmaceutical therapy to surgery may be needed to prevent more serious conditions.
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Adrenocortical carcinoma, though very rare, are cancerous and aggressive tumors of the adrenal cortex. It is most common in children under the age of 10 and in adults between the ages of 50 and 60. Over-production of hormone due to this type of tumor may lead to high blood pressure, weight gain and diabetes. Unfortunately, most adults may not present with symptoms at all, making diagnosis challenging.
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While it’s true that many patients do not have any symptoms associated with adrenocortical tumors, changes in general health can offer important clues. Increasing blood pressure, unexplained weight gain and even the development of diabetes may point to these kinds of tumors.
Depending on the disease, simple blood and urine tests can sometimes detect the presence of excessive adrenal hormones, indicating a potential tumor. If a tumor is detected, CT scans, MRIs and nuclear medicine scans, as well as radiological testing can help pinpoint masses.
Many adrenal tumors are now found incidentally during radiologic tests being performed for other reasons (CT scans, PET scans and ultrasound).
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Surgery is the best way to cure adrenal cancer. Advances in laparoscopic surgery enable us to more easily and successfully remove an adrenal gland or tumor. Three-to-four small incisions (each just one-half to one inch in length) are all that is needed to remove the mass. This approach shortens hospital stays, speeds recovery and reduces the possibility of long-term complications due to surgery. At times, especially if dealing with cancer of the adrenal, a more traditional open surgery is performed.
Here at Vanderbilt, we were one of the first in the country to remove adrenal tumors from the back, avoiding surgery in the abdominal area. See story on our posterior retroperitoneoscopic surgery.
Patients with adrenocortical carcinoma may receive radiation or chemotherapy following surgery to reduce the risk of recurrence.
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Pheochromocytomas are rare, slow growing tumors in the adrenal gland that over-produce adrenaline. Because adrenaline controls our natural “fight or flight response,” over-production of this hormone can lead to high blood pressure, headaches, sweating and heart palpitations.
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Biochemical tests are performed to assess the level of adrenaline and related hormones in blood and/or urine. If levels are high, a CAT scan or MRI should be performed to locate the tumor. Other tests that might be used include nuclear medicine tests such as MIBG scans and PET scans.
Though the tumor may not be cancerous, too much adrenaline can cause serious health conditions, including heart attack and stroke, which is why it is critically important to surgically remove these tumors once detected.Laparoscopic approaches (3 or 4 small incisions) are commonly used in most patients. Some patients, especially those with large tumors, may require open surgery.
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Paragangliomas are rare tumors that develop in nerves outside the brain and spinal cord. They can be found anywhere along the body’s major arteries, but are most commonly found in the abdomen. Like pheochromocytomas, paragangliomas are tumors that over-produce adrenaline, so similar symptoms and serious health conditions present, including heart attack and stroke.
But unlike pheochromocytomas, paragangliomas have a higher incidence of malignancy – as high as 40 to 50% in some studies. Patients with pheochromocytomas and paragangliomas may have a genetic condition that can be passed down to their offspring, leading to formation of the same tumors.
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Because over-production of adrenaline can lead to heart attack and stroke, it is very important to surgically remove these tumors once detected.