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


Geroula, Aikaterini; Deutschbein, Timo; Langton, Katharina; Masjkur, Jimmy Rusdian; Pamporaki, Christina; Peitzsch, Mirko; Fliedner, Stephanie; Timmers, Henri J; Bornstein, Stefan R; Beuschlein, Felix; Stell, Anthony; Januszewicz, Andrzej; Prejbisz, Aleksander; Fassnacht, Martin; Lenders, Jacques; Eisenhofer, Graeme (2019). Pheochromocytoma and paraganglioma: Clinical feature based disease probability in relation to catecholamine biochemistry and reason for disease suspicion. European Journal of Endocrinology, 181(4):409-420.

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 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 body mass index (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.

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 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 body mass index (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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Endocrinology and Diabetology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:1 October 2019
Deposited On:05 Sep 2019 11:22
Last Modified:06 Oct 2019 06:01
Publisher:BioScientifica Ltd.
ISSN:0804-4643
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1530/EJE-19-0159
PubMed ID:31370000

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