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


METHODS
All patients (n = 121) who had been hospitalized and treated for PC/PG over 47 years.


METHODS
Retrospective review of patients with PC/PG regarding presenting symptoms, tumour characteristics, clinical management and long-term outcome after treatment.


METHODS
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.


CONCLUSIONS
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.
Khorram-Manesh
A
A
Lundberg Laboratory for Cancer Research, Sahlgrenska University Hospital, Göteborg, Sweden.
Ahlman
H
H
Nilsson
O
O
Friberg
P
P
Odén
A
A
Stenström
G
G
Hansson
G
G
Stenquist
O
O
Wängberg
B
B
Tisell
L-E
LE
Jansson
S
S
D000310:Adrenal Gland Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D000313:Adrenal Medulla / Q000473:pathology; D000328:Adult; D000368:Aged; D001794:Blood Pressure / Q000502:physiology; D005260:Female; D006801:Humans; D006965:Hyperplasia; D006973:Hypertension / Q000150:complications; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010235:Paraganglioma / Q000401:mortality / Q000473:pathology / Q000601:surgery; D010673:Pheochromocytoma / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D011184:Postoperative Period; D011300:Preoperative Care / Q000379:methods; D011942:Receptors, Adrenergic, alpha / Q000008:administration & dosage; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
D011942:Receptors, Adrenergic, alpha