Subscribe to RSS
DOI: 10.1055/s-0032-1331217
Mortality Associated with Phaeochromocytoma
Publication History
received 03 August 2012
accepted 13 November 2012
Publication Date:
15 January 2013 (online)
Abstract
Two major categories of mortality are distinguished in patients with phaeochromocytoma. First, the effects of excessive circulating catecholamines may result in lethal complications if the disease is not diagnosed and/or treated timely. The second category of mortality is related to development of metastatic disease or other neoplasms. Improvements in disease recognition and diagnosis over the past few decades have reduced mortality from undiagnosed tumours. Nevertheless, many tumours remain unrecognised until they cause severe complications. Death resulting from unrecognised or untreated tumour is caused by cardiovascular complications. There are also numerous drugs and diagnostic or therapeutic manipulations that can cause fatal complications in patients with phaeochromocytoma. Previously it has been reported that operative mortality was as high as 50% in unprepared patients with phaeochromocytoma who were operated and in whom the diagnosis was unsuspected. Today mortality during surgery in medically prepared patients with the tumour is minimal. Phaeochromocytomas may be malignant at presentation or metastases may develop later, but both scenarios are associated with a potentially lethal outcome. Patients with phaeochromocytoma run an increased risk to develop other tumours, resulting in an increased mortality risk compared to the general population. Phaeochromocytoma during pregnancy represents a condition with potentially high maternal and foetal mortality. However, today phaeochromocytoma in pregnancy is recognised earlier and in conjunction with improved medical management, maternal mortality has decreased to less than 5%.
-
References
- 1 Fraenkel F. Ein Fall von doppelsteitigem völlig latent verlaufenem Nebennierentumor und gleichzeitiger Nephritis mit Veränderungen am Circulationsapparat und Retinitis. Arch Pathol Anat Physiol Klin Med 1886; 103: 244-263
- 2 Manger WM, Gifford Jr RW. Clinical and experimental pheochromocytoma. Second ed. Cambridge, MA: Blackwell Science; 1996
- 3 Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011; 29: 2049-2060
- 4 Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc 1981; 56: 354-360
- 5 Hartley L, Perry-Keene D. Phaeochromocytoma in Queensland – 1970 – 83. Aust N Z J Surg 1985; 55: 471-475
- 6 Zelinka T, Petrak O, Turkova H, Holaj R, Strauch B, Krsek M, Vrankova AB, Musil Z, Duskova J, Kubinyi J, Michalsky D, Novak K, Widimsky J. High incidence of cardiovascular complications in pheochromocytoma. Horm Metab Res 2012; 44: 379-384
- 7 Timmers HJ, Brouwers FM, Hermus AR, Sweep FC, Verhofstad AA, Verbeek AL, Pacak K, Lenders JW. Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma. Endocr Relat Cancer 2008; 15: 1127-1133
- 8 Caputo C, Fishbane S, Shapiro L, Courgi RG, Kostadinov S, Donovan V, Epstein D. Pheochromocytoma multisystem crisis in a patient with multiple endocrine neoplasia type IIB and pyelonephritis. Am J Kidney Dis 2002; 39: E23
- 9 Gordon DL, Atamian SD, Brooks MH, Gattuso P, Castelli MJ, Valaitis J, Thomas Jr W. Fever in pheochromocytoma. Arch Intern Med 1992; 152: 1269-1272
- 10 Kizer JR, Koniaris LS, Edelman St JD, John Sutton MG. Pheochromocytoma crisis, cardiomyopathy, and hemodynamic collapse. Chest 2000; 118: 1221-1223
- 11 Moran ME, Rosenberg DJ, Zornow DH. Pheochromocytoma multisystem crisis. Urology 2006; 67: 846 e819-e820
- 12 Newell KA, Prinz RA, Pickleman J, Braithwaite S, Brooks M, Karson TH, Glisson S. Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg 1988; 123: 956-959
- 13 Eisenhofer G, Rivers G, Rosas AL, Quezado Z, Manger WM, Pacak K. Adverse drug reactions in patients with phaeochromocytoma: incidence, prevention and management. Drug Saf 2007; 30: 1031-1062
- 14 Smith DS, Aukburg SJ, Levitt JD. Induction of anesthesia in a patient with an undiagnosed pheochromocytoma. Anesthesiology 1978; 49: 368-369
- 15 Walker T, Bail DH, Schmid E, Haeberle L, Kuehne H, Ziemer G. Challenging the advanced: cardiac surgery without awareness of a pheochromocytoma. Thorac Cardiovasc Surg 2006; 54: 498-499
- 16 Sheaves R, Chew SL, Grossman AB. The dangers of unopposed beta-adrenergic blockade in phaeochromocytoma. Postgrad Med J 1995; 71: 58-59
- 17 Sibal L, Jovanovic A, Agarwal SC, Peaston RT, James RA, Lennard TW, Bliss R, Batchelor A, Perros P. Phaeochromocytomas presenting as acute crises after beta blockade therapy. Clin Endocrinol (Oxf) 2006; 65: 186-190
- 18 Brody IA. Shock after administration of prochlorperazine in patient with pheochromocytoma; report of a case with spontaneous tumor destruction. J Am Med Assoc 1959; 169: 1749-1752
- 19 Plouin PF, Menard J, Corvol P. Hypertensive crisis in patient with phaeochromocytoma given metoclopramide. Lancet 1976; 2: 1357-1358
- 20 Takai Y, Seki H, Kinoshita K. Pheochromocytoma in pregnancy manifesting hypertensive crisis induced by metoclopramide. Int J Gynaecol Obstet 1997; 59: 133-137
- 21 Jan T, Metzger BE, Baumann G. Epinephrine-producing pheochromocytoma with hypertensive crisis after corticotropin injection. Am J Med 1990; 89: 824-825
- 22 Moorhead 2nd EL, Caldwell JR, Kelly AR, Morales AR. The diagnosis of pheochromocytoma. Analysis of 26 cases. JAMA 1966; 196: 1107-1113
- 23 Rosas AL, Kasperlik-Zaluska AA, Papierska L, Bass BL, Pacak K, Eisenhofer G. Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature. Eur J Endocrinol 2008; 158: 423-429
- 24 Yi DW, Kim SY, Shin DH, Kang YH, Son SM. Pheochromocytoma crisis after a dexamethasone suppression test for adrenal incidentaloma. Endocrine 2010; 37: 213-219
- 25 Takahashi N, Shimada T, Tanabe K, Yoshitomi H, Murakami Y, Ishibashi Y, Kikkawa R, Yano S, Araki A, Inoue A. Steroid-induced crisis and rhabdomyolysis in a patient with pheochromocytoma: a case report and review. Int J Cardiol 2011; 146: e41-e45
- 26 Rashid-Farokhi F, Cheraghvandi A, Masjedi MR. Pheochromocytoma crisis due to glucocorticoid administration: a case report and review of the literature. Arch Iran Med 2009; 12: 190-194
- 27 Minamori Y, Yammoto M, Tanaka A, Takeda N, Yasuda K. Hazard of glucagon test in diabetic patients. Hypertensive crisis in asymptomatic pheochromocytoma. Diabetes Care 1992; 15: 1437-1438
- 28 Schorr RT, Rogers SN. Intraoperative cardiovascular crisis caused by glucagon. Arch Surg 1987; 122: 833-834
- 29 Eisenhofer G, Bornstein SR. Surgery: Risks of hemodynamic instability in pheochromocytoma. Nat Rev Endocrinol 2010; 6: 301-302
- 30 Apgar V, Papper EM. Pheochromocytoma. Anesthetic management during surgical treatment. AMA Arch Surg 1951; 62: 634-648
- 31 Modlin IM, Farndon JR, Shepherd A, Johnston ID, Kennedy TL, Montgomery DA, Welbourn RB. Phaeochromocytomas in 72 patients: clinical and diagnostic features, treatment and long term results. Br J Surg 1979; 66: 456-465
- 32 Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G. Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center. J Clin Endocrinol Metab 2001; 86: 1480-1486
- 33 Bruynzeel H, Feelders RA, Groenland TH, van den Meiracker AH, van Eijck CH, Lange JF, de Herder WW, Kazemier G. Risk Factors for Hemodynamic Instability during Surgery for Pheochromocytoma. J Clin Endocrinol Metab 2010; 95: 678-685
- 34 Walz MK, Alesina PF, Wenger FA, Koch JA, Neumann HP, Petersenn S, Schmid KW, Mann K. Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients. World J Surg 2006; 30: 899-908
- 35 Amar L, Baudin E, Burnichon N, Peyrard S, Silvera S, Bertherat J, Bertagna X, Schlumberger M, Jeunemaitre X, Gimenez-Roqueplo AP, Plouin PF. Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas. J Clin Endocrinol Metab 2007; 92: 3822-3828
- 36 Grogan RH, Mitmaker EJ, Duh QY. Changing paradigms in the treatment of malignant pheochromocytoma. Cancer Control 2011; 18: 104-112
- 37 Scholz T, Eisenhofer G, Pacak K, Dralle H, Lehnert H. Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab 2007; 92: 1217-1225
- 38 Wangberg B, Muth A, Khorram-Manesh A, Jansson S, Nilsson O, Forssell-Aronsson E, Tisell L, Ahlman H. Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci 2006; 1073: 512-516
- 39 Weismann D, Fassnacht M, Schubert B, Bonfig R, Tschammler A, Timm S, Hahner S, Wunder C, Allolio B. A dangerous liaison – pheochromocytoma in patients with malignant disease. Ann Surg Oncol 2006; 13: 1696-1701
- 40 Plouin PF, Fitzgerald P, Rich T, Ayala-Ramirez M, Perrier ND, Baudin E, Jimenez C. Metastatic pheochromocytoma and paraganglioma: focus on therapeutics. Horm Metab Res 2012; 44: 390-399
- 41 Eisenhofer G, Lenders JW, Siegert G, Bornstein SR, Friberg P, Milosevic D, Mannelli M, Linehan WM, Adams K, Timmers HJ, Pacak K. Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer 2012; 48: 1739-1749
- 42 Timmers HJ, Taieb D, Pacak K. Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma. Horm Metab Res 2012; 44: 367-372
- 43 Eisenhofer G, Schott M, Bornstein S. Pheochromocytoma and paraganglioma: recent progress and new vistas for improved patient care. Horm Metab Res 2012; 44: 325-327
- 44 Eisenhofer G, Tischler AS, de Krijger RR. Diagnostic tests and biomarkers for pheochromocytoma and extra-adrenal paraganglioma: from routine laboratory methods to disease stratification. Endocr Pathol 2012; 23: 4-14
- 45 Khorram-Manesh A, Ahlman H, Nilsson O, Friberg P, Oden A, Stenstrom G, Hansson G, Stenquist O, Wangberg B, Tisell LE, Jansson S. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med 2005; 258: 55-66
- 46 Pasini B, Stratakis CA. SDH mutations in tumorigenesis and inherited endocrine tumours: lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med 2009; 266: 19-42
- 47 Khorram-Manesh A, Ahlman H, Nilsson O, Oden A, Jansson S. Mortality associated with pheochromocytoma in a large Swedish cohort. Eur J Surg Oncol 2004; 30: 556-559
- 48 Almog B, Kupferminc MJ, Many A, Lessing JB. Pheochromocytoma in pregnancy – a case report and review of the literature. Acta Obstet Gynecol Scand 2000; 79: 709-711
- 49 Botchan A, Hauser R, Kupfermine M, Grisaru D, Peyser MR, Lessing JB. Pheochromocytoma in pregnancy: case report and review of the literature. Obstet Gynecol Surv 1995; 50: 321-327
- 50 Brunt LM. Phaeochromocytoma in pregnancy. Br J Surg 2001; 88: 481-483
- 51 Burgess 3rd GE. Alpha blockade and surgical intervention of pheochromocytoma in pregnancy. Obstet Gynecol 1979; 53: 266-270
- 52 Harper MA, Murnaghan GA, Kennedy L, Hadden DR, Atkinson AB. Phaeochromocytoma in pregnancy. Five cases and a review of the literature. Br J Obstet Gynaecol 1989; 96: 594-606
- 53 Lau P, Permezel M, Dawson P, Chester S, Collier N, Forbes I. Phaeochromocytoma in pregnancy. Aust N Z J Obstet Gynaecol 1996; 36: 472-476
- 54 Leak D, Carroll JJ, Robinson DC, Ashworth EJ. Management of pheochromocytoma during pregnancy. Can Med Assoc J 1977; 116: 371-375
- 55 Oliva R, Angelos P, Kaplan E, Bakris G. Pheochromocytoma in pregnancy: a case series and review. Hypertension 2010; 55: 600-606
- 56 Sam S, Molitch ME. Timing and special concerns regarding endocrine surgery during pregnancy. Endocrinol Metab Clin North Am 2003; 32: 337-354
- 57 Schenker JG, Granat M. Phaeochromocytoma and pregnancy – an updated appraisal. Aust N Z J Obstet Gynaecol 1982; 22: 1-10
- 58 Manger WM. The vagaries of pheochromocytomas. Am J Hypertens 2005; 18: 1266-1270
- 59 Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol 2012; 166: 143-150