: Phaeochromocytoma and paraganglioma (PPGL) are chromaffin cell tumours that require timely diagnosis because of their potentially serious cardiovascular and sometimes life- threatening sequelae. Tremendous progress in biochemical testing, imaging, genetics and pathophysiological understanding of the tumours has far-reaching implications for physicians dealing with hypertension and more importantly affected patients. Because hypertension is a classical clinical clue for PPGL, physicians involved in hypertension care are those who are often the first to consider this diagnosis. However, there have been profound changes in how PPGLs are discovered; this is often now based on incidental findings of adrenal or other masses during imaging and increasingly during surveillance based on rapidly emerging new hereditary causes of PPGL. We therefore address the relevant genetic causes of PPGLs and outline how genetic testing can be incorporated within clinical care. In addition to conventional imaging (computed tomography, MRI), new functional imaging approaches are evaluated. The novel knowledge of genotype-phenotype relationships, linking distinct genetic causes of disease to clinical behaviour and biochemical phenotype, provides the rationale for patient-tailored strategies for diagnosis, follow-up and surveillance. Most appropriate preoperative evaluation and preparation of patients are reviewed, as is minimally invasive surgery. Finally, we discuss risk factors for developing metastatic disease and how they may facilitate personalised follow-up. Experts from the European Society of Hypertension have prepared this position document that summarizes the current knowledge in epidemiology, genetics, diagnosis, treatment and surveillance of PPGL.

Aix Marseille Université Department of Endocrinology Institut National de la Santé et de la Recherche Médicale Marseille France

Assistance Publique Hôpitaux de Paris AP HP Hôpital Européen Georges Pompidou Service de Génétique Université de Paris PARCC INSERM Paris France

Center for Hypertension 3rd Department of Medicine Division of Endocrinology and Metabolism 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Department of Endocrinology University of Groningen University Medical Center Groningen Groningen The Netherlands

Department of Experimental and Clinical Biomedical Sciences University of Florence Florence Italy

Department of General Visceral and Transplantation Surgery Section of Endocrine Surgery University of Duisburg Essen Essen Germany

Department of Hypertension Institute of Cardiology Warsaw Poland

Department of Internal Medicine Radboud University Medical Center Nijmegen The Netherlands

Department of Medical Sciences Uppsala University Uppsala Sweden

Department of Medicine 3 University Hospital Carl Gustav Carus Technical University Dresden Dresden Germany

Department of Nuclear Medicine La Timone University Hospital European Center for Research in Medical Imaging Aix Marseille University Marseille France

Division of Endocrinology and Diabetes Department of Internal Medicine 1 University Hospital University of Würzburg Würzburg Germany

Hereditary Endocrine Cancer Group Human Cancer Genetics Program Spanish National Cancer Research Centre Madrid Spain

Institute of Clinical Chemistry and Laboratory Medicine Medical Faculty Carl Gustav Carus Technical University Dresden Dresden Germany

Section on Medical Neuroendocrinology Eunice Kennedy Shriver National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA

Unité d'Hypertension Artérielle Hôpital Européen Georges Pompidou Assistance Publique Hôpitaux de Paris Université de Paris PARCC INSERM Paris France

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Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement

Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options

Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants

Single-nuclei and bulk-tissue gene-expression analysis of pheochromocytoma and paraganglioma links disease subtypes with tumor microenvironment

Deep Membrane Proteome Profiling Reveals Overexpression of Prostate-Specific Membrane Antigen (PSMA) in High-Risk Human Paraganglioma and Pheochromocytoma, Suggesting New Theranostic Opportunity