Pheochromocytoma Essay

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Pheochromocytoma is a rare tumor which is formed in chromaffin cells of the adrenal glands. Pheochromocytomas are also known as adrenal chromaffin tumors (1). Chromaffin cells are found in the medulla of the adrenal glands (Figure 1). The adrenal glands located just above the kidneys. These cells receive neuronal messages from the brain via neurotransmitters and, as a result of these messages, release hormones in to the bloodstream (Figure 2). The main catecholamines produced in the adrenal glands are dopamine, norepinephrine, and epinephrine (also known as adrenaline) (1). Pheochromocytomas cause the adrenal glands to make too much of these hormones, which are called catecholamines. Adrenal glands with pheochromocytomas produce excess catecholamines, which can cause high blood pressure or hypertension (1). High blood pressure that goes untreated can lead to a variety of severe health problems including heart disease and stroke. Typical symptoms of pheochromocytomas include headaches, excessive sweating, pounding of the heart, pain in the chest, and a feeling of anxiety (1).
Pheochromocytomas can be to be inherited and several gene have been identified, that when mutated, lead to pheochromocytoma (Figure 3). The majority of inherited pheochromocytomas are due to mutations in the genes VHL, RET, NF1, and MAX. Pheochromocytoma has been associated with Von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN 2), and neurofibromatosis type 1 (NF1). Von Hippel-Lindau disease (VHL) is a condition caused by the mutation of the gene VHL, where cysts and tumors grow on the brain, the kidneys, and adrenal glands. Neurofibromatosis 1 (NF1) is a condition where tumors develop on the skin and optic nerve and is caused by ...

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... known as alpha blockers. Drugs known as tyrosine kinase inhibitors available can also be used to prevent the formation of hormones by the tumor. In some cases, radiation therapy (using high- dose x-rays or other high-energy rays to kill cancer cells) or chemotherapy (using drugs to kill cancer cells) may be used to treat pheochromocytoma (4).
In general, pheochromocytomas found in the adrenal gland are benign, and can be successfully removed by surgery (4). Patients with recurring pheochromocytoma tumors, tumors that have metastasized or that cannot be removed surgically have outlooks that are fair to poor. Both malignant and benign pheochromocytomas can recur after surgical removal of the tumor. Therefore, long-term follow-up care is very important after surgery to keep the outlooks fair to good with additional medicinal or surgical treatments as appropriate (4).

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