Pheochromocytomas detected by biochemical screening in predisposed subjects are associated with lower prevalence of clinical and biochemical manifestations and smaller tumors than pheochromocytomas detected by signs and symptoms.
BACKGROUND
Sporadic pheochromocytomas are detected by clinical signs and symptoms, whereas pheochromocytomas in patients with a known hereditary predisposition for these tumors are detected by repetitive screening for catecholamine excess.


OBJECTIVE
To document the clinical, biochemical, and pathological differences between patients with sporadic pheochromocytomas, detected by signs and symptoms and patients with pheochromocytomas, detected by biochemical screening in established hereditary syndromes.


METHODS
Retrospective follow-up study.


METHODS
We included 60 consecutive patients diagnosed with pheochromocytoma (pheochromocytomas detected by signs and symptoms: n=28 and pheochromocytomas detected by screening: n=32) in our center.


RESULTS
Patients with pheochromocytomas detected by screening presented with less complaints of diaphoresis (P<0.01), palpitations (P=0.01), paleness (P=0.01), nausea (P<0.01), and vomiting (P=0.01) compared with patients with symptomatic pheochromocytomas. Patients with pheochromocytomas detected by screening tended to be younger at the time of diagnosis (41+/-2 vs 47+/-3 years, P=0.07). In addition, patients with pheochromocytomas detected by screening had significantly lower rates of 24-h urinary catecholamine excretion, and considerably smaller tumors (3.7+/-0.5 vs 7.3+/-0.7 cm, P<0.01).


CONCLUSIONS
Pheochromocytomas detected by screening of patients with a hereditary predisposition have a much lower prevalence of signs and symptoms, lower catecholamine excess, and smaller tumors, compared with sporadic pheochromocytomas, detected by signs and symptoms. These data support the benefits of screening for pheochromocytomas in patients with hereditary syndromes predisposing for these tumors.
van Duinen
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Department of Endocrinology and Metabolic Diseases, Center of Human and Clinical Genetics, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands. n.van_duinen@lumc.nl
Steenvoorden
D
D
Bonsing
B A
BA
Vuyk
J
J
Vriends
A H J T
AH
Jansen
J C
JC
Romijn
J A
JA
Corssmit
E P M
EP
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000453:epidemiology*; D000328:Adult; D000368:Aged; D006801:Humans; D008875:Middle Aged; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000453:epidemiology* / Q000473:pathology; D012189:Retrospective Studies