Pheochromocytoma: Review

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Table of Contents

Introduction
Pathophysiology of Pheochromocytoma
Assessment of Patient
Causes
Nursing Diagnosis
Treatment
Prevention
Conclusion
References

Introduction

Pheochromocytoma is one of the diseases affecting various people globally; hence, it is important that its treatment methods be known clearly. Generally, pheochromocytoma is a condition or disease that results from failure of extra adrenal tissue or due to a neuroendocrine tumor of the medulla of the adrenal glands, causing secretion with high amount of catecholamine, especially the norepinephrine and epinephrine; it is also known as high blood pressure or hypertension (Sweeney & Griffing, 2011). This disease originates from the chromaffin cells of the adrenal glands. Generally, pheochromocytoma knows no age when it attacks, as any person may suffer from this condition; however, the most common incidences of pheochromocytoma occur between the age of 30 years and the age of 50 years, and only ten percent occur in children. In extreme cases, there are reports of the pheochromocytoma condition occurring in newborns that are barely a week old and aged people nearing their centenary year. Unfortunately, this condition may result into heart diseases; indeed, the malignant cases are always fatal. Although research has found out that this condition is common in adults between 30 and 40 years of age (Sweeney & Griffing, 2011), the condition is more common in women than in men. Therefore, it is important to understand this disease and identify proper and better options for its treatment and prevention (Sweeney & Griffing, 2011).

Pathophysiology of Pheochromocytoma

Generally, pheochromocytoma refers to a condition that forms a cancerous-like lump adrenal gland tissue. Normally, adrenal glands secrete hormones such as “aldosterone, testosterone, cortisol, norepinephrine, and epinephrine,” which have various functions in the body including “to regulate important bodily functions such as blood pressure, stress, blood sugar, immune response, metabolism, and heart rate” (Krause and Roth, 2012). Therefore, inadequacy or lack of these hormones can lead to ill health. Generally, when adrenal glands are interrupted in their functioning, they tend to be inconsistent in production or secretion of hormones; this hormonal imbalance may be fatal, especially when excess of norepinephrine and epinephrine is secreted in acute pheochromocytoma. Although the condition tends not to be fatal when compared with cancer, it can be very disturbing when great hormonal imbalance is detected. About 80% cases of this condition always occur in the adrenal glands and about 15% occurs outside the adrenal glands (Garg, Kharb, Brar, Gundgurthi & Mittal, 2011).

When pheochromocytoma occurs outside the glands, it is called paragangliomas and it result in the release of catecholamine, that is, epinephrine, norepinephrine and dopamine. These hormones control heart rate, metabolism, and blood pressure; indeed, paragangliomas condition will occur when there is increased production of these hormones, resulting to fatal problems if not treated immediately. In addition, problems such as “high blood pressure tend to increase the risk of heart diseases and stroke” (Jack, 2007).

Pheochromocytoma is always associated with irregular supply of oxygen to the tissues, a condition referred to as hypoxia. Chromaffin cells form the main dwelling and breeding place for pheochromocytoma, where secretion of adrenaline is interrupted. A big number of tumors arising from these cells are less harmful, as they tend to be dormant or just dedicated in a specific area without spreading beyond the adrenal glands; however, if there is delay in treatment, they may cause many problems including death. In most cases, it is difficult to detect pheochromocytoma where chromaffin cells are absent, thus explaining the reason why this condition normally affects the adrenal glands as this is the area where chromaffin cells are concentrated. However, pheochromocytoma condition normally inhabits the medulla, leading to excess production of adrenaline, which later leads to severe and adverse conditions such as hypertension. Indeed, Keiser has emphasized this by postulating that, the pheochromocytoma condition causes the “adrenal medulla to secrete too much adrenaline, which in turn causes high blood pressure,” and this later causes the other symptoms of the disease (Keiser, 2008).

Assessment of Patient

Patient assessment findings may include the presence of hemodynamic parameters, fluid and electrolyte status, for example, intake of coffee and tea. It is also important to examine a patient to establish whether there is bleeding or possibility of injuries resulting from severe high blood pressure.

As discussed above, pheochromocytoma is a condition associated with hormonal imbalance, where excess adrenaline is produced. Therefore, in diagnosing the condition, the technician will look high levels of catecholamine hormone or adrenergic crisis (AC), both of which are known to cause hypertension and tachycardia conditions respectively (Barzon & Marco, 2012). Indeed, excess of the above hormones will be clear symptoms of the presence of pheochromocytoma.

Those who suffer from pheochromocytoma always have three classic symptoms, which include, “headache, sweating, and heart palpitations (a fast heart beat) in association with markedly elevated blood pressure” (Barzon & Marco, 2012). Other main symptoms of the pheochromocytoma disease include but not limited to “skin sensations, flank pain, elevated heart rate, palpitations, anxiety often resembling that of a panic attack, diaphoresis (excessive sweating), headaches( most common symptom), pallor, sudden onset of severe headaches, sweating, abdominal pain, high blood pressure, rapid heart rate, irritability, anxiety, nausea, tremors, and weight loss” (Barzon & Marco, 2012).

It is also important to note that, in some cases, some people never develop symptoms of this disease and about “10% of cases are discovered incidentally, meaning that they are not suspected and only found when the patient is undergoing diagnostic studies for other conditions” (Barzon & Marco, 2012).

Causes

Generally, pheochromocytoma was always believed to occur by itself, but current studies show that it can be inherited and passed through from parents to their children. In most cases, patients who get this disease through inheritance have also been found to suffer from other genetic disorders. These disorders may include “Von Hippel-Lindau disease (VHL) – a condition in which cysts and tumors grow in the central nervous system, on the brain , kidneys, adrenal glands, or other areas of the body” (Krause and Roth, 2012). Again, there is neurofibromatosis 1 (NF1), which is the development of tumors on “skin (neurofibromas), pigmented skin spots, and tumors of the optic nerve” (Keiser, 2008). Lastly, there is multiple endocrine neoplasia type II (MEN), which is a form of thyroid cancer (Keiser, 2008). Other common causes of pheochromocytoma condition include drugs such as corticosteroids because they affect the adrenal glands; chemotherapy agents are sometimes believed to cause pheochromocytoma. In addition, the use of surgical anesthesia can cause this disease, mainly because this anesthesia always leads to tumors and affects tissues associated with adrenal glands. Moreover, emotional stress is also another cause of pheochromocytoma, as it always causes high blood pressure. Another cause of pheochromocytoma is thyroid cancer, which blocks the oxygen supply to the adrenal glands. Lastly, tremors and genetic diseases affecting the genes may cause this condition. This again will cause problems to the adrenal glands leading to pheochromocytoma, (Barzon & Marco, 2012).

Nursing Diagnosis

There are various nursing diagnoses associated with pheochromocytoma, among them being hypertensive crisis, hypermetabolism, anxiety, and ineffective renal tissue perfusion. All these occur to a patient at varying degrees and if not well managed or prevented, they may become fatal to the patient. This section of the paper will discuss two nursing diagnosis, which are ineffective cardiopulmonary tissue perfusion and anxiety related to potential complications of pheochromocytoma. To begin with, ineffective cardiopulmonary tissue perfusion associated with pheochromocytoma may result due to low oxygen brought about by impaired circulation. The nurse needs to check for altered respiratory rate, abnormal arterial blood gases, nasal flaring, chest pain, and chest retraction among others. In addition, the nurse should observe the time taken to refill capillary as well as the use of accessory muscles. Close monitoring is therefore important, especially due to the fact that cardiopulmonary tissue perfusion may lead to fatal results.

The second nursing diagnosis is anxiety that results from potential complications of pheochromocytoma. Generally, a patient may be anxious after learning about presence of pheochromocytoma, and when this problem is not prevented, it may lead to high blood pressure, which has adverse effects as discussed above. Moreover, anxiety may lead to accelerated nonvagal adverse events, especially for patients known to experience cardiac problems.

The above diagnoses may be intervened through various means. First, intervention for ineffective cardiopulmonary tissue perfusion may include allowing the patient to have a bed rest with elevated head position. In addition, the nurse should assess the blood pressure and monitor arterial pressure, including measuring the input and output. Moreover, the nurse should educate the patient about avoidance of fatigue as well as compliance with doctor’s advice on medication and fluid intake. On anxiety diagnosis, interventions may include reassuring and calming the patient. In addition, the patients should be allowed to identify and express their feelings about the diagnosis, and with the help of a nurse, be allowed to perform activities that reduce anxiety. Moreover, the nurse will need to demonstrate to the patient, methods for positively coping pheochromocytoma.

Treatment

There are various drugs that can be used to treat pheochromocytoma, however, the most effective drugs include alpha-blockers, beta-blockers, calcium channel blockers, and antihypertensives, as these drugs are always used to prepare patients effectively before surgery is done to remove the tumors affecting the adrenal glands (Ahmed, 2007). However, the best treatment for this condition always involves surgery of the tumor by either open laparotomy or laparoscopy, especially with the help of endocrine and anesthesia resources. This section of the paper will discuss two drugs, one being an alpha-blocker and the other one a beta-blocker.

One alpha-blocker drug used in treatment of pheochromocytoma is phenoxybenzamine (usually traded as Dibenzyline), which has a slow-down effect on hypertension or high blood pressure associated with pheochromocytoma. Generally, phenoxybenzamine acts as an adrenergic blocking agent by controlling the adrenaline from stimulating the muscles on the walls of blood vessels (small arteries and veins), thus causing a relaxation effect. The relaxation of these muscles enables the blood vessels to remain open and normalize blood circulation, thereby lowering the blood pressure. This drug should only be used under the prescription a doctor, and once administered, it should be taken consistently even when signs of sickness are not felt, as sudden stop may be fatal. However, phenoxybenzamine causes various side effects, some of which may be serious while others are mild and less harmful. The most common side effects of this drug are nausea and stomach upset. However, when a patient realizes severe side effects such as fainting and increased heartbeat, urgent medical consultation is advisable.

The second drug used in treatment of pheochromocytoma is Normodyne (normally marketed as labetalol), which is a beta-blocker. Normally, labetalol is used in control of hypertension due to its effect of lowering heartbeat and enhancing blood circulation. However, before taking labetalol, the patient must have first received treatment using an alpha-blocker drug. One of the side effects of this drug is dizziness, which comes as a spinning sensation in the mind thus interfering with thinking and reaction. Therefore, a patient should avoid situations that require alertness immediately after taking the drug.

Prevention

It is unfortunate that little is known about pheochromocytoma prevention and this is due to environmental factors and the fact that it an inheritance disease; therefore, it cannot be easily prevented (Jack, 2007). Despite that, it is important that the patients keep proper balance diet, avoid stress, and if possible, avoid smoking and taking other drugs including stimulants like coffee and tea. Again, proper precaution measures and self-care can be used to prevent the disease. Lastly, it is important that the patients be taught to adopt life long lifestyle skills to reduce the severe effects of the disease (Ahmed, 2007).

Conclusion

In conclusion, we have discussed about pheochromocytoma and found out that a tumor affects the adrenal glands, leading production of excess adrenaline that causes hypertension. Again, the discussion has established the symptoms of pheochromocytoma, which include headache, anxiety, and abdominal pain, and fast breathing problems among others. In addition, some of the causes discussed above include the fact that the condition is in most cases genetically inherited. Besides, pheochromocytoma may be treated through various surgical procedures but the most effective are open laparotomy and laparoscopy. Lastly, the discussion has established that, since this condition is inherited, it does not have a direct prevention method that can be used, but there are various methods that can be used to reduce its occurrence including eating balance diet and avoiding drugs and alcoholic substances.

References

Ahmed, A. (2007). Preoperative Management of Pheochromocytoma: Anesthetic Implications. Journal of Pakistan Medical Association, 57 (3), 140-147.

Barzon, L. & Marco, B. (2012). Diagnosis and Management of Adrenal Incidentalomas. Journal of Urology, 163 (2): Pp. 398-407.

Garg, M. K. et al. (2011). Medical management of pheochromocytoma: Role of the endocrinologist. Indian Journal of Endocrinology and Metabolism, 15 (4), S329-S336.

Jack, K. (2007). Preoperative management of the pheochromocytoma patient. The Journal of Clinical Endocrinology & Metabolism, 92 (11): pp.45.

Keiser, H. R. (2008). Pheochromocytoma and Related Tumors. New York: Oxford University press.

Krause, L. and Roth, E. (2012). Pheochromocytoma.Health line Network Inc. Web.

Sweeney, A.T. & Griffing, G. T. (2011). Pheochromocytoma. London: Oxford University Press.

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