Some tumors caused by MEN 2 are malignant. Pheochromocytomas are cancerous tumors of the adrenal gland, which overproduce the hormone adrenaline. They usually form in the adrenal medulla, the central portion of the adrenal gland. Adrenaline helps the body regulate blood pressure and manage stress.
Symptoms
The first sign of pheochromocytoma is severely high blood pressure, caused by the overproduction of adrenaline. Patients may also experience headaches, excessive sweating or anxiousness. Other symptoms include irregular heartbeat, nervous shaking, chest pain, nausea, weight loss or an inability to tolerate heat.
Anyone with these symptoms or problems related to high blood pressure should be screened for pheochromocytoma. These include patients whose high blood pressure is hard to control, those who require more than four blood pressure medications and patients whose condition appeared before age 35 or after age 60.
Diagnosis
Pheochromocytoma is diagnosed using urine and blood tests. Urine tests measure the types of hormones produced by the adrenal gland - adrenaline, norepinephrine and dopamine. The concentration of these hormones in the urine indicates the presence of pheochromocytoma. Blood tests measure the level of the hormone catecholamine.
A CT scan or MRI (magnetic resonance imaging) may be used to confirm the presence of pheochromocytoma. Computed tomography, or a CT scan, consists of an X-ray that takes pictures of the body from many different angles. Then, a computer will consolidate all of these images into a detailed, 3-dimensional view of the inside of the body. Sometimes a special dye is injected to help provide better detail. Magnetic resonance imaging, or MRI, uses magnetic fields, and not X-rays, to produce images.
Treatment
Pheochromocytomas are usually removed surgically. Radiation may be used to shrink inoperable tumors, reducing the amount of adrenaline produced.
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