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

Author:

Geroula Aikaterini1,Deutschbein Timo2,Langton Katharina1,Masjkur Jimmy1,Pamporaki Christina1,Peitzsch Mirko3,Fliedner Stephanie4,Timmers Henri J L M5,Bornstein Stefan R1,Beuschlein Felix67,Stell Anthony8,Januszewicz Andrzej9,Prejbisz Aleksander9,Fassnacht Martin2,Lenders Jacques W M15,Eisenhofer Graeme13

Affiliation:

1. 1Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

2. 2Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital of Würzburg, Würzburg, Germany

3. 3Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

4. 4First Department of Medicine, University Medical Center Schleswig-Holstein, Lübeck, Germany

5. 5Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands

6. 6Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany

7. 7Department of Endocrinology, Diabetology and Clinical Nutrition, UniviersitätsSpital Zürich, Zürich, Switzerland

8. 8Department of Computing and Information, University of Melbourne, Melbourne, Australia

9. 9Department of Hypertension, Institute of Cardiology, Warsaw, Poland

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.

Publisher

Bioscientifica

Subject

Endocrinology,General Medicine,Endocrinology, Diabetes and Metabolism

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