A Review And Analysis Of Pheochromocytoma

1491 words - 6 pages

Introduction:
Pheochromocytoma is a rare condition affecting approximately five out of every one million people (0.0005%) of the population. On average, there are 1000 cases diagnosed per year in the United States. Pheochromocytomas are neuroendocrine tumors that are responsible for an up-regulation of the entire sympathetic nervous system (SNS). These tumors reside mostly in the medulla and adrenal glands; however, some do arise in extra-adrenal chromaffin tissue (Zelinka 2011). Pheochromocytoma generally refers to the tumors within the medulla and adrenal glands, while the extra-adrenal areas are referred to as paraganglioma. Common locations for paraganglioma consist of the heart, along the spinal cord, back wall of the abdomen, the neck, and the bladder. Pheochromocytomas originate in chromaffin cells, which are responsible for producing catecholamines. Therefore, pheochromocytomas secrete high levels of catecholamines, primarily norepinephrine along with a lesser amount of epinephrine. Norepinephrine is the primary stimulant of the sympathetic nervous system, thus this explains how these tumors can cause near-constant activation of the SNS resulting in a wide array of symptoms. The majority of pheochromocytomas are benign tumors (~90%); however, they can be malignant and are vastly more troublesome if they are. Interestingly, extra-adrenal pheochromocytomas (paragangliomas) are the most likely to become malignant tumors. Most cases have no identifiable cause, but up to 25% of cases are being attributed to heredity. The only treatment method currently available for a patient with pheochromocytoma(s) is surgery. Both benign and malignant tumors are necessary to remove as the patient’s blood pressure is in jeopardy with these neuroendocrine tumors. A large issue with surgery though is that specific care and procedure must be taken as a stressful situation can release catecholamines. Any increase to the patient’s already elevated levels of catecholamines before, during, or after the surgery could have dire consequences. As proposed by Zelinka et al., any tumor that is five centimeters (or larger) should be monitored very closely as it is at a high risk of achieving metastatic spreading. Additionally, malignant tumors may require chemotherapy or radiation treatment following the surgery to eradicate remaining sections of the tumor due to metastasis. Unfortunately, both benign and malignant tumors have a recurrence rate of ~10%. Thus, long-term care and observation is critical to give the patient the best possible outcome/future. People within the ages of 30 to 50 are the most likely to develop pheochromocytomas, although they have been present in individual as young as five days or as old as 92 years. Interestingly, people who develop a pheochromocytoma with no family history of risk factors for developing pheochromocytomas are more likely to be diagnosed at an early age. Pheochromocytomas tend to be present in individuals that are already...

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