Pheochromocytoma is a tumor that grows in the center of the adrenal gland. There are two adrenal glands in the body above each kidney. Adrenal glands are responsible for the production of hormones that monitor and regulate almost every organ and tissue in the body. A pheochromocytoma can produce excess hormones that can increase heart rate and blood pressure. This can be life threatening if left unidentified or unrecognized. This tumor usually begins to develop between the age of forty and sixty, but can develop at any age. Treatment for this tumor can remediate high blood pressure and heart rate.
Symptoms of pheochromocytoma are rapid heart rate, irregular heart rate, extreme sweating, pain in the chest, pain in the upper abdomen, hand tremors, anxiety, extreme fear and pallor. The most common symptom is high blood pressure. The tumor can create excessive levels of the hormone adrenaline causing the fight or flight reflex to occur in the patient - as noted in the symptoms above. The excess hormone can be produced in bursts or continuously - which cause continuous hypertension (high blood pressure) or extreme variances in hypertension. When the adrenaline is being released in bursts, the body usually reacts with the same symptoms each time, developing a specific pattern of signs or symptoms.
A high blood pressure attach is known as a hypertensive crisis. This attack can occur during physical activity or other events within the patient’s body. The onset of attacks can occur from the following: lifting, exercising, changing body placement or position, defecating, emotional upset, anxiety or when receiving anesthesia.
External triggers can lead to a hypertensive crisis, such as smoking and eating foods rich n tyramine - such as beer, wine and cheese. Tyramine is an amino acid that enables the body to regulate blood pressure. Hypertensive crises can occur several times a week and can last from fifteen to sixty minutes per episode.
There are a few tests that can be used to diagnose pheochromocytoma. Blood, urine tests and images/scans are used to diagnose this tumor of the adrenal gland. Blood tests and urine tests will determine the levels of the hormones. The hormones that are checked for are adrenaline and noradrenaline, these are known as epinephrine and norepinephrine respectively. Another substance that is checked for is the breakdown or by-products called metanephrines. High levels of these substances indicate the possibility of pheochromocytoma.
If the hormone levels are high in the blood and urine, the next step will be a scan of the kidneys to determine if a tumor is present and the location of the tumor. CT scan, magnetic resonance imaging and an m-iodobenzylguanidine (also known as an MIBG) scan can be used to detect a tumor. Other areas besides the abdomen may be scanned, such as the neck, pelvis or chest.
Adrenal gland tumors are often discovered accidentally during imaging for other symptoms. A majority of adrenal gland tumors are non cancerous and asymptomatic. Most tumors require observation and little or no treatment.
Most of the pheochromocytomas do not have a known case. Ten to twenty percent are due to an inherited risk, found in four different syndromes: Multiple Endocrine Neoplasia type 2A and 2B, von Hippel Lindau disease and Neurofibromatosis type 1. These disorders can be inherited by men and women. Parents have a fifty percent risk of passing on these diseases to their children. Before testing, genetic counseling is strongly suggested.
On the cellular level, paragangliomas are masses that grown in sympathetic neural tissue. Chromaffin cells are part of the make up of paragangliomas, mostly due to the higher levels of adrenaline, epinephrine and dopamine. Most paragangliomas, almost ninety percent occur on the adrenal gland and are pheochromocytomas.
Only ten percent of cases are malignant. Untreated pheochromocytoma can be fatal because of the extremely high blood pressure. The five year survival rate from the time of diagnosis is 43%.
Treatment for pheochromocytomas includes a combination of surgery, radiation therapy and chemotherapy. Surgery is the number one approach to treatment for pheochromocytoma. Chemotherapy is used to kill off any remaining cancer in the body. Radiation therapy is also used to kill any cancer cells that are in the body or treat painful site where the cancer has metastasized. Radiation is hardly ever used to treat the tumor itself, surgery is the best option.
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