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CNE: Pheochromocytoma: A rare tumor in adults and children

Through assessment and education, we can help ensure that the long-term effects of critical illness are recognized, addressed, and perhaps averted.


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After reading the article you should be able to:

1. Identify signs and symptoms of pheochromocytoma.
2. Discuss nursing and medical management for a patient with pheochromocytoma.
3. Develop a plan of care for a patient with pheochromocytoma.

Statement of Financial Disclosure for “Pheochromocytoma: A rare tumor in adults and children”:
RN's editorial staff, including Patricia Lederman, Martha K. Raymond, RN, BSN, James Fraleigh, and Catherine Radwan, have no relationships to disclose. Managing Editor Steve Mullett reports that he is a stockholder in Wellpoint, Inc., Pfizer, Inc., and American Oriental Bioengineering, Inc. Editor Kathleen A. Moore, RN, BS, has no financial relationships to disclose. Kay Ball, RN, MSA, CNOR, FAAN has reviewed this article and reports that she is a consultant with AHC Media LLC and a stockholder with STERIS Corp.



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Originally Posted June 2008

By EDEN TSEGAY, RN, MS, FNP-C; GRACE ANYANGO, RN, MS, FNP-C; SHARON L. VAN SELL, RN, EdD, PAHM; and MARILYN ILLERANDERSON, RN, C, PhD

EDEN TSEGAY and GRACE ANYANGO are family nurse practitioners at Parkland Health & Hospital System in Dallas. SHARON VAN SELL is professor of Texas Women’s University in Dallas and a member of the RNeditorial board. At the time this manuscript was submitted, MARILYN MILLER-ANDERSON was associate clinical professor of nursing at TWU in Dallas. The authors have no financial relationships to disclose. EDITOR: KATHLEEN A. MOORE, RN, BS.

This rare, life-threatening tumor requires quick recognition and intervention, including aggressive preoperative treatment to reduce mortality.

Helen Potter, 48, is admitted to your telemetry floor for hypertensive crisis and a three-day history of severe headaches. She has been taking metoprolol (Lopressor) 50 mg twice a day for high blood pressure, which was diagnosed six months ago. A computed tomography (CT) scan done prior to her arrival on your unit from the ED has ruled out subarachnoid hemorrhage. The results of her blood work are pending.

Upon her arrival, you place Ms. Potter on continuous cardiac and blood pressure monitoring. Her vital signs are BP 205/100 mm Hg; HR 120 beats per minute, sinus tachycardia; respirations 22 per minute; and temperature 98.6° F (37° C). You administer labetalol (Normodyne, Trandate) 10 mg IV push, as ordered, but her blood pressure remains high. And you notify the physician, who orders repeated doses; these, too, fail to keep her BP below the desired parameter of 180/100 mm Hg.

Ms. Potter complains of palpitations and nausea after returning from the bathroom. She's restless, anxious, and sweating profusely, and her BP is now 225/110 mm Hg. You check her blood sugar by finger stick. It's 130 mg/dL, within normal range. You call the doctor again, and he asks you to draw Ms. Potter's blood for plasma-free metanephrines. Suspected diagnosis: pheochromocytoma.

A closer look at pheochromocytomas

A pheochromocytoma, in most cases, is a catecholamine-secreting tumor of the adrenal medulla. This type of tumor typically arises from the chromaffin tissue of the interior adrenal gland. In 10% of patients, however, pheochromocytomas are extra-adrenal, meaning they arise in other areas of the body—in some instances, outside of the abdomen.1 Extra-adrenal pheochromocytomas are also referred to as paragangliomas.

Pheochromocytomas are rare, with only five to eight cases diagnosed per million people a year.1,2 They occur most frequently in adults in their 30s and 40s.3 Ten percent are in children and adolescents,4 most commonly in 11- and 12-year-olds.3

Approximately nine out of 10 pheochromocytomas are benign.4 Most arise sporadically, rather than genetically or by inheritance. Between 10% and 15% of cases are familial.1

Though the presentation of pheochromocytoma varies, hypertension occurs in 90% of patients and may be sustained or paroxysmal.1,2 In adults, blood pressure is apt to increase suddenly, with periodic elevations. Other common signs and symptoms for this patient population include diaphoresis and headaches, described as intense and global. Children with pheochromocytoma are more likely to present with nausea and vomiting, and their hypertension tends to be sustained.5

Be aware, though, that not all patients with pheochromocytoma will have elevated blood pressure. In some, BP will be normal or decreased. In elderly patients, physiological changes and coexisting disease may obscure the signs of pheochromocytoma, making diagnosis even more challenging.6

What causes blood pressure to climb

The signs and symptoms of pheochromocytoma are caused by excess circulating catecholamines.4 Pheochromocytomas secrete the catecholamines norepinephrine, epinephrine, and dopamine. Norepinephrine, an alpha-adrenergic agonist, causes vasoconstriction, reflex bradycardia, sweating, pupillary dilation, impaired gastrointestinal motility, and decreased insulin secretion. Epinephrine acts on beta-1 receptors, raising heart rate and augmenting myocardial contraction, thereby increasing systolic blood pressure, cardiac output, and oxygen consumption. At high levels, dopamine increases heart rate; at lower levels, it can raise systolic pressure and cardiac output.

Levels of the catecholamine metabolites metanephrine and normetanephrine are almost always elevated in patients with a pheochromocytoma. Therefore, the workup begins with biochemical testing of blood or urine for measurement of these substances.7 Recent guidelines from the National Comprehensive Cancer Network recommend testing for either plasma-free meta-nephrine and normetanephrine, or for urine metanephrine, catecholamines, creatinine, and perhaps dopamine.7 Equivocal results may necessitate additional biochemical testing.

Other tests you might see ordered include a comprehensive metabolic panel (CMP), complete blood count (CBC), erythrocyte sedimentation rate (ESR), plasma rennin, and tests of thyroid function (T4, FT4, T3, and TSH). Excessive catecholamine secretion can lead to hypermetabolism, hyperglycemia, elevated ESR, and increased plasma renin activity. Thyroid function, however, should remain normal.1

Once biochemical testing reveals increased catecholamine levels, CT and magnetic resonance imaging (MRI) of the chest and abdomen are used to locate the site of the tumor and evaluate its size. When biochemical tests are positive but the scans reveal no mass, a metaiodobenzylguanidine (MIBG) nuclear medicine scan may be successful in locating the lesion.4,7 The National Institutes of Health Clinical Center is recruiting patients with pheochromocytoma for a study to develop better methods of diagnosing and treating the condition. For more information, go to www.clinicaltrials.gov/ct/show/NCT00004847.

Preoperative treatment reduces mortality

Surgery is the treatment of choice for pheochromocytoma;8 the goal being to remove the entire adrenal gland or tumor, or as much of the tumor as possible. Laparoscopic removal has advantages over conventional laparotomy in that it reduces postoperative morbidity, requires a shorter hospital stay, and is less expensive.9

Pharmacological blockade to reduce catecholamine production, initiated well before surgery, is essential to preventing serious intra operative complications, including hypertensive crisis, cardiac arrhythmias, pulmonary edema, and myocardial ischemia.9,10 Perioperative blood pressure control is so crucial to reducing mortality that, even in the most severe cases, pretreatment followed by elective surgery is preferable to more urgent tumor resection without proper pretreatment.9

Aggressive control of hypertension should begin 10 days before surgery with alpha-adrenergic blockers. These drugs prevent norephinephrine-induced constriction of the small blood vessels. Typically, patients receive phenoxybenzamine (Dibenzyline), starting at 10 mg twice a day. The physician or nurse practitioner then increases the dose by 10 to 20 mg every two to three days to a maximum of 1 mg/kg/day. Another option is doxazosin (Cardura), administered in increasing doses, from 1 to 16 mg, once a day. Other alpha-adrenergic blockers include prazosin (Minipress) and terazosin (Hyrtrin).

After a few days, once the alpha blockade has lowered blood pressure, the physician may add a beta blocker, such as propranolol (Inderal) or atenolol (Tenormin), particularly if the patient has a rapid heart beat or irregular rhythm. These drugs decrease heart rate and contractility, reduce myocardial oxygen demand, and lower blood pressure.

Adequate treatment and control of blood pressure before, during, and after tumor resection drastically reduces the surgical mortality rate.9 Blockade of beta-adrenoceptors should never precede blockade of alpha-adrenoceptors, however, as this could result in hypertensive crisis.4,7,9

To assure that blood pressure is well controlled during surgery, clinicians have proposed several criteria. The patient's BP should be below 160/90 mm Hg for at least 24 hours. Orthostatic hypotension should occur, but blood pressure while the patient is upright shouldn't drop below 84/40 mm Hg. In addition, the patient should have no more than one ventricular extra-systolic beat in a five-minute period, and no ST segment changes or T wave inversions on his EKG for one week prior to surgery.9

Managing hypertension after surgery

Two major postoperative complications of pheochromocytoma resection are severe hypotension and hypoglycemia. Therefore, part of your postoperative nursing care will consist of monitoring blood pressure and glucose levels. Hypotension develops after surgery because of the sudden fall in circulating catecholamines that occurs once the tumor is removed, and because of the continuing presence of alpha-adrenoceptor blockade. If hypotension occurs, you'll administer fluids. Occasionally, patients will also receive intravenous ephedrine. In addition to hypotension, glucose levels may fall as insulin is released following tumor removal.9

Along with blood sugar and BP, you'll monitor the patient's other vital signs, hemodynamic parameters, fluid and electrolyte status—including intake and urinary output—and urine catecholamine levels. You'll assess the patient for bleeding and infection, which are rare,8 and for pain. Instruct patients not to strain or make rapid position changes. Administer medication to reduce pain and anxiety, which can trigger a hypertensive crisis.8

When the tumor is cancerous

About 10% of pheochromocytomas are malignant,4 a condition for which there is no cure. Radical surgical removal of the tumor aims to improve symptoms and survival rates. Chemo therapy, though not a cure, is sometimes tried. Radiation therapy is indicated to relieve symptoms of metastatic pheo chromocytoma.8 Common sites of metastasis include the bones, lungs, liver, and lymph nodes. Early detection may prevent metastasis.

Based on biopsy results of the resected tumor, it is impossible to predict whether a patient will develop malignant dis-ease.9 In general, patients with tumors larger than 5 cm and tumors located outside of the adrenal glands have a higher risk of malignant disease than those with smaller tumors and tumors located in the adrenal gland itself.9 When malignancy is diagnosed, the course of the disease is highly individualized, with patients living an average of five years after diagnosis.9

Follow-up is long-term

After 10 days of pretreatment, Ms. Potter undergoes surgery without significant complications. Her tumor, detected on MRI, was small and confined to her left adrenal gland. She is now normotensive.

When preparing her for discharge, you explain that pheochromocytomas can recur, and that the prognosis is more favorable when tumors are detected early. You also explain that she'll need to follow up with her doctor at least twice a year for the next few years, and then annually until instructed otherwise.7 Extra-adrenal and hereditary pheochromoctyomas may require lifelong follow-up.9

If not recognized early on and treated appropriately, pheochromocytomas can cause irreversible—even fatal—cardiovascular and cerebrovascular complications and other long-term, end-organ damage.11 Suspect this tumor in patients with labile hypertension or with sudden increases in blood pressure accompanied by other classic signs: palpitations, headache, perspiration, and pallor.12 Also consider it when patients have marked hypertension that is resistant to treatment.2 Your awareness of pheochromocytoma's signs and symptoms will help ensure that this rare but dangerous tumor doesn't go undetected and that, once identified, it is treated promptly and appropriately.


References

1. Fitzgerald, P. A. (2006). Endocrinology. In Tierney, L. M., McPhee, S. J. & Papadakis, M. A. (Eds.), Current medical diagnosis and treatment, 2006 (45th ed.). (pp. 1098-1193). New York: McGraw-Hill.

2. Kudva, Y. C., Sawka, A. M., & Young, W. F. (2003). The laboratory diagnosis of adrenal pheochromocytoma: The Mayo Clinic experience. J Clin Endocrin Metab, 88(10), 4533.

3. Ciftci, A. O., Tanyel, F. C., et al. (2001). Pheochromocytoma in children. J Ped Surg, 36(3), 447.

4. Sweeney, A. T. "Pheochromocytoma." 2007. http://www.emedicine.com/med/topic.htm (12 May 2008).

5. Pozo, J., Munoz, M. T., et al. (2005). Sporadic phaeochromocytoma in childhood: Clinical and molecular variability. J Pediatr Endocrinol Metab, 18(6), 527.

6. Bravo, M. L., & Tagle, R. (2003). Pheochromocytoma: State of the art and future prospects. Endocrine Reviews, 24(4), 539.

7. National Comprehensive Cancer Network. "NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors." 2008. http://www.nccn.org/professionals/physicians_gls/PDF/neuroendrocrine.pdf (12 May 2008).

8. Goodman, M., & Wickham, R. (2005). Endocrine malignancies. In Yarbro, H., Frogge, M. H., & Goodman, M., Cancer nursing: Principles and practice (6th ed.). Sudbury, MA: Jones & Bartlett Publishers.

9. Lenders, J. W., Eisenhofer, G., et al. (2005). Pheochromocytoma. Lancet, 366, 665.

10. Khorram-Manesh, A., Ahlman, H., et al. (2005). Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med, 258(1), 55.

11. Manger, W. M. (2003). Editorial: In search of pheochromocytoma. J Clin Endocrin & Metab, 88(9), 4080.

12. The Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC 7). The Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC 7): The Guidelines. (2003). 1206. Hypertension, 42, 1206.


Signs and symptoms of pheochromocytoma

  • Hypertension, sustained or paroxysmal
  • Palpitations
  • Headaches
  • Excessive sweating
  • Pallor or flushing
  • Nausea, vomiting
  • Anxiety, panic
  • Weight loss
  • Postural hypotension
  • Hyperglycemia

The rule of 10s

Pheochromocytomas roughly follow a rule of 10s. About 10% of cases:

  • arise outside of the adrenal glands
  • are not accompanied by hypertension
  • are bilateral
  • occur in children
  • are familial
  • are malignant
  • recur
  • are associated with Multiple Endocrine Neoplasia (MEN) Syndromes
  • present with a stroke

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