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Long-term outcome of a large series of patients surgically treated for pheochromocytoma

Journal article
Authors Amir Khorram-Manesh
Håkan Ahlman
Ola Nilsson
Peter Friberg
A. Oden
G. Stenstrom
G. Hansson
Ola Stenqvist
Bo Wängberg
Lars-Eric Tisell
Svante Jansson
Published in Journal of internal medicine
Volume 258
Issue 1
Pages 55-66
ISSN 0954-6820 (Print)
Publication year 2005
Published at Cardiovascular Institute
Institute of Laboratory Medicine, Dept of Pathology
Institute of Surgical Sciences, Department of Surgery
Institute of Surgical Sciences, Department of Anaesthesiology and Intensive Care
Pages 55-66
Language en
Keywords Adrenal Gland Neoplasms/mortality/pathology/*surgery, Adrenal Medulla/pathology, Adult, Aged, Blood Pressure/physiology, Female, Humans, Hyperplasia, Hypertension/complications, Male, Middle Aged, Neoplasm Invasiveness, Paraganglioma/mortality/pathology/surgery, Pheochromocytoma/mortality/pathology/*surgery, Postoperative Period, Preoperative Care/methods, Receptors, Adrenergic, alpha/administration & dosage, Retrospective Studies, Survival Analysis, Treatment Outcome
Subject categories Medical and Health Sciences


OBJECTIVE: To analyse the morbidity, mortality and long-term outcome in a consecutive series of surgically treated patients with pheochromocytoma (PC), or paraganglioma (PG), from the western region of Sweden between 1950 and 1997. PATIENTS: All patients (n = 121) who had been hospitalized and treated for PC/PG over 47 years. DESIGN: Retrospective review of patients with PC/PG regarding presenting symptoms, tumour characteristics, clinical management and long-term outcome after treatment. SETTING: One referral centre for all patients from the western region of Sweden. RESULTS: During an observation of 15 +/- 6 years, 42 patients died vs. 23.6 expected in the general population (P < 0.001). There was no intra- or post-operative mortality. Four patients with sporadic disease died of malignant PC and six with hereditary disease of associated neuroectodermal tumours. Five patients died of other malignancies, 20 of cardiovascular disease and seven of other causes. Besides older age at primary surgery, elevated urinary excretion of methoxy-catecholamines was the only observed risk factor for death (P = 0.02). At diagnosis 85% of the patients were hypertensive; one year after surgery more than half were still hypertensive. However, pre- and post-operative hypertension did not influence the risk for death versus controls. CONCLUSION: Pheochromocytoma/PG can be safely treated by surgery. Death of malignant PC/PG was unusual, but the patients as a group had an increased risk of death. We recommend life-long follow-up of patients treated for PC/PG with screening for recurrent tumour in sporadic cases and for associated tumours in hereditary cases. This strategy would also be helpful in diagnosing cardiovascular disease at an early stage.

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