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Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion.

Abstract：

Objective:Hypertension and symptoms of catecholamine excess are features of pheochromocytomas and paragangliomas (PPGLs). This prospective observational cohort study assessed whether differences in presenting features in patients tested for PPGLs might assist establishing likelihood of disease. Design and methods:Patients were tested for PPGLs because of signs and symptoms, an incidental mass on imaging or routine surveillance due to previous history or hereditary risk. Patients with (n = 245) compared to without (n = 1820) PPGLs were identified on follow-up. Differences in presenting features were then examined to assess the probability of disease and relationships to catecholamine excess. Results:Hyperhidrosis, palpitations, pallor, tremor and nausea were 30-90% more prevalent (P < 0.001) among patients with than without PPGLs, whereas headache, flushing and other symptoms showed little or no differences. Although heart rates were higher (P < 0.0001) in patients with than without PPGLs, blood pressures were not higher and were positively correlated to BMI, which was lower (P < 0.0001) in patients with than without PPGLs. From these differences in clinical features, a score system was established that indicated a 5.8-fold higher probability of PPGLs in patients with high than low scores. Higher scores among patients with PPGLs were associated, independently of tumor size, with higher biochemical indices of catecholamine excess. Conclusions:This study identifies a complex of five signs and symptoms combined with lower BMI and elevated heart rate as key features in patients with PPGLs. Prevalences of these features, which reflect variable tumoral catecholamine production, may be used to triage patients according to likelihood of disease.

journal_name

Eur J Endocrinol

journal_title

European journal of endocrinology

authors

Geroula A,Deutschbein T,Langton K,Masjkur J,Pamporaki C,Peitzsch M,Fliedner S,Timmers HJLM,Bornstein SR,Beuschlein F,Stell A,Januszewicz A,Prejbisz A,Fassnacht M,Lenders JWM,Eisenhofer G

doi

10.1530/EJE-19-0159

subject

Has Abstract

pub_date

2019-10-01 00:00:00

pages

409-420

issue

eissn

0804-4643

issn

1479-683X

pii

EJE-19-0159.R2

journal_volume

181

pub_type

临床试验,杂志文章,多中心研究

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Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion.