Horm Metab Res 2013; 45(02): 154-158
DOI: 10.1055/s-0032-1331217
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© Georg Thieme Verlag KG Stuttgart · New York

Mortality Associated with Phaeochromocytoma

A. Prejbisz
1   Department of Hypertension, Institute of Cardiology, Warsaw, Poland
,
J.W. M. Lenders
2   Department of Internal Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
3   Institute of Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
G. Eisenhofer
3   Institute of Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
A. Januszewicz
1   Department of Hypertension, Institute of Cardiology, Warsaw, Poland
› Author Affiliations
Further Information

Publication History

received 03 August 2012

accepted 13 November 2012

Publication Date:
15 January 2013 (online)

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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%.