Aikaterini Geroula1, Timo Deutschbein2, Katharina Langton1, Jimmy Masjkur1, Christina Pamporaki1, Mirko Peitzsch3, Stephanie Fliedner4, Henri J L M Timmers5, Stefan R Bornstein1, Felix Beuschlein6,7, Anthony Stell8, Andrzej Januszewicz9, Aleksander Prejbisz9, Martin Fassnacht2, Jacques W M Lenders1,5, Graeme Eisenhofer1,3. 1. Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. 2. Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital of Würzburg, Würzburg, Germany. 3. Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. 4. First Department of Medicine, University Medical Center Schleswig-Holstein, Lübeck, Germany. 5. Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands. 6. Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany. 7. Department of Endocrinology, Diabetology and Clinical Nutrition, UniviersitätsSpital Zürich, Zürich, Switzerland. 8. Department of Computing and Information, University of Melbourne, Melbourne, Australia. 9. Department 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.
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.
Authors: Minghao Li; Christina Pamporaki; Stephanie M J Fliedner; Henri J L M Timmers; Svenja Nölting; Felix Beuschlein; Aleksander Prejbisz; Hanna Remde; Mercedes Robledo; Stefan R Bornstein; Jacques W M Lenders; Graeme Eisenhofer; Nicole Bechmann Journal: Discov Oncol Date: 2021-03-19
Authors: R Garcia-Carbonero; F Matute Teresa; E Mercader-Cidoncha; M Mitjavila-Casanovas; M Robledo; I Tena; C Alvarez-Escola; M Arístegui; M R Bella-Cueto; C Ferrer-Albiach; F A Hanzu Journal: Clin Transl Oncol Date: 2021-05-06 Impact factor: 3.405