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Pheochromocytoma Multisystem Crisis: A Surgical Emergency

Pheochromocytoma Multisystem Crisis: A Surgical Emergency Abstract • Three of 27 patients treated for pheochromocytoma between 1974 and 1987 presented with pheochromocytoma multisystem crisis (PMC). This unusual presentation consists of multiple organ system failure, temperature often greater than 40°C, encephalopathy, and hypertension and/or hypotension. Although urgent medical therapy achieved blood pressure control in all three patients, the other manifestations of PMC progressed rapidly in spite of alpha and even beta blockade. The first patient died during attempts to localize a septic focus. The other two patients underwent urgent adrenalectomy and had postoperative improvement in their multiple organ system failure. All three tumors were large and produced markedly elevated levels of epinephrine. In conclusion (1) PMC is an unusual presentation of pheochromocytoma; (2) its manifestations include multiple organ system failure, high fever, encephalopathy, and vascular lability; (3) it may result from increased epinephrine secretion; and (4) successful treatment of PMC demands prompt diagnosis, vigorous medical preparation, and emergency tumor removal if the patient's condition continues to deteriorate. (Arch Surg 1988;123:956-959) References 1. Hamrin B: Sustained hypotension and shock due to an adrenaline-secreting pheochromocytoma . Lancet 1962;2:123-124.Crossref 2. Fred HL, Allred DP, Garber HE, et al: Pheochromocytoma masquerading as overwhelming infection . Am Heart J 1967;73:149-154.Crossref 3. Richmond J, Frazer SC, Millar DR: Paroxysmal hypotension due to an adrenaline-secreting pheochromocytoma . Lancet 1961;2:904-906.Crossref 4. Delaney JP, Paritzky AZ: Necrosis of a pheochromocytoma with shock . N Engl J Med 1979;280:1394-1395.Crossref 5. Atuk NO, Teja K, Mondzelewski JP, et al: A vascular necrosis of pheochromocytoma followed by spontaneous remission . Arch Intern Med 1977;137:1073-1075.Crossref 6. French C, Campagna FA: Pheochromocytoma with shock, marked leukocytosis, and unusual electrocardiograms: Case report and review of the literature . Ann Intern Med 1961;55:127-134.Crossref 7. Kahn MT, Mullon DA: Pheochromocytoma without hypertension . JAMA 1964;188:74-75.Crossref 8. Page LB, Raker JW, Berberick FR: Pheochromocytoma with predominant epinephrine secretion . Am J Med 1969;47:648-652.Crossref 9. Leather HM, Shaw DB, Cates JE: Six cases of pheochromocytoma with unusual clinical manifestations . Br Med J 1962;1:1373-1378.Crossref 10. Case 6-1986, Case records of the Massachusetts General Hospital: Weekly clinicopathological exercises . N Engl J Med 1986;314:431-439.Crossref 11. Radtke WE, Kazmier FJ, Rutherford BD, et al: Cardiovascular complications of pheochromocytoma crisis . Am J Cardiol 1975;35:701-705.Crossref 12. Hill JB, Schwartzman RJ: Cerebral infarction and disseminated intravascular coagulation with pheochromocytoma . Arch Neurol 1981; 38:395.Crossref 13. Bornemann M, Hill SC, Kidd GS II: Lactic acidosis in pheochromocytoma . Ann Intern Med 1986;105:880-882.Crossref 14. Munk Z, Tolis G, Jones W, et al: Pheochromocytoma presenting with pulmonary edema and hyperamylasemia . Can Med Assoc J 1977;116:357-359. 15. Northfield TC: Cardiac complications of pheochromocytoma . Br Heart J 1967;29:588-593.Crossref 16. Sode J, Getzen LC, Osborne DP: Cardiac arrythmias and cardiomyopathy associated with pheochromocytoma . Am J Surg 1967;114:927-931.Crossref 17. Imperato-McGinley J, Gautier T, Ehlers K, et al: Reversibility of catecholamine-induced dilated cardiomyopathy in a child with a pheochromocytoma . N Engl J Med 1987;316:793-797.Crossref 18. Van Vliet PD, Burchell HB, Titus JL: Focal myocarditis associated with pheochromocytoma . N Engl J Med 1966;274:1102-1108.Crossref 19. Freier DT, Eckhauser FE, Harrison TS: Pheochromocytoma: A persistently problematic and still potentially lethal disease . Arch Surg 1980;115:388-391.Crossref 20. Cubeddu L, Zarate NA, Rosales CB, et al: Prazosin and propranolol in preoperative management of pheochromocytoma . Clin Pharmacol Ther 1982;32:156-160.Crossref 21. Hauptman JB, Modlinger RS, Entel NH: Pheochromocytoma resistant to adrenergic blockade . Arch Intern Med 1983;143:2321-2323.Crossref 22. Aronoff SL, Passamani E, Borowshy BA, et al: Norepinephrine and epinephrine secretion from a clinically epinephrine-secreting pheochromocytoma . Am J Med 1980;69:321-324.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1988 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1988.01400320042007
Publisher site
See Article on Publisher Site

Abstract

Abstract • Three of 27 patients treated for pheochromocytoma between 1974 and 1987 presented with pheochromocytoma multisystem crisis (PMC). This unusual presentation consists of multiple organ system failure, temperature often greater than 40°C, encephalopathy, and hypertension and/or hypotension. Although urgent medical therapy achieved blood pressure control in all three patients, the other manifestations of PMC progressed rapidly in spite of alpha and even beta blockade. The first patient died during attempts to localize a septic focus. The other two patients underwent urgent adrenalectomy and had postoperative improvement in their multiple organ system failure. All three tumors were large and produced markedly elevated levels of epinephrine. In conclusion (1) PMC is an unusual presentation of pheochromocytoma; (2) its manifestations include multiple organ system failure, high fever, encephalopathy, and vascular lability; (3) it may result from increased epinephrine secretion; and (4) successful treatment of PMC demands prompt diagnosis, vigorous medical preparation, and emergency tumor removal if the patient's condition continues to deteriorate. (Arch Surg 1988;123:956-959) References 1. Hamrin B: Sustained hypotension and shock due to an adrenaline-secreting pheochromocytoma . Lancet 1962;2:123-124.Crossref 2. Fred HL, Allred DP, Garber HE, et al: Pheochromocytoma masquerading as overwhelming infection . Am Heart J 1967;73:149-154.Crossref 3. Richmond J, Frazer SC, Millar DR: Paroxysmal hypotension due to an adrenaline-secreting pheochromocytoma . Lancet 1961;2:904-906.Crossref 4. Delaney JP, Paritzky AZ: Necrosis of a pheochromocytoma with shock . N Engl J Med 1979;280:1394-1395.Crossref 5. Atuk NO, Teja K, Mondzelewski JP, et al: A vascular necrosis of pheochromocytoma followed by spontaneous remission . Arch Intern Med 1977;137:1073-1075.Crossref 6. French C, Campagna FA: Pheochromocytoma with shock, marked leukocytosis, and unusual electrocardiograms: Case report and review of the literature . Ann Intern Med 1961;55:127-134.Crossref 7. Kahn MT, Mullon DA: Pheochromocytoma without hypertension . JAMA 1964;188:74-75.Crossref 8. Page LB, Raker JW, Berberick FR: Pheochromocytoma with predominant epinephrine secretion . Am J Med 1969;47:648-652.Crossref 9. Leather HM, Shaw DB, Cates JE: Six cases of pheochromocytoma with unusual clinical manifestations . Br Med J 1962;1:1373-1378.Crossref 10. Case 6-1986, Case records of the Massachusetts General Hospital: Weekly clinicopathological exercises . N Engl J Med 1986;314:431-439.Crossref 11. Radtke WE, Kazmier FJ, Rutherford BD, et al: Cardiovascular complications of pheochromocytoma crisis . Am J Cardiol 1975;35:701-705.Crossref 12. Hill JB, Schwartzman RJ: Cerebral infarction and disseminated intravascular coagulation with pheochromocytoma . Arch Neurol 1981; 38:395.Crossref 13. Bornemann M, Hill SC, Kidd GS II: Lactic acidosis in pheochromocytoma . Ann Intern Med 1986;105:880-882.Crossref 14. Munk Z, Tolis G, Jones W, et al: Pheochromocytoma presenting with pulmonary edema and hyperamylasemia . Can Med Assoc J 1977;116:357-359. 15. Northfield TC: Cardiac complications of pheochromocytoma . Br Heart J 1967;29:588-593.Crossref 16. Sode J, Getzen LC, Osborne DP: Cardiac arrythmias and cardiomyopathy associated with pheochromocytoma . Am J Surg 1967;114:927-931.Crossref 17. Imperato-McGinley J, Gautier T, Ehlers K, et al: Reversibility of catecholamine-induced dilated cardiomyopathy in a child with a pheochromocytoma . N Engl J Med 1987;316:793-797.Crossref 18. Van Vliet PD, Burchell HB, Titus JL: Focal myocarditis associated with pheochromocytoma . N Engl J Med 1966;274:1102-1108.Crossref 19. Freier DT, Eckhauser FE, Harrison TS: Pheochromocytoma: A persistently problematic and still potentially lethal disease . Arch Surg 1980;115:388-391.Crossref 20. Cubeddu L, Zarate NA, Rosales CB, et al: Prazosin and propranolol in preoperative management of pheochromocytoma . Clin Pharmacol Ther 1982;32:156-160.Crossref 21. Hauptman JB, Modlinger RS, Entel NH: Pheochromocytoma resistant to adrenergic blockade . Arch Intern Med 1983;143:2321-2323.Crossref 22. Aronoff SL, Passamani E, Borowshy BA, et al: Norepinephrine and epinephrine secretion from a clinically epinephrine-secreting pheochromocytoma . Am J Med 1980;69:321-324.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: Aug 1, 1988

References