Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

3rd Department of Medicine Department of Endocrinology and Metabolism of the 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Centro de Investigación Biomédica en Red de Enfermedades Raras Madrid Spain

Clinical Research Center Ijinkai Takeda General Hospital Kyoto Japan

Clinical Research Institute of Endocrinology and Metabolism National Hospital Organization Kyoto Medical Center and Endocrine Center Kyoto Japan

Columbia University Irving Medical Center New York City NY USA

Department of Diabetes Endocrinology and Metabolism National Center for Global Health and Medicine Tokyo Japan

Department of Endocrine Surgery Aix Marseille University Conception Hospital Marseille France

Department of Endocrinology Diabetology and Clinical Nutrition University Hospital Zurich and University of Zurich Zurich Switzerland

Department of Endocrinology Royal North Shore Hospital and Cancer Genetics Laboratory Kolling Institute University of Sydney Sydney New South Wales Australia

Department of Endocrinology Seth GS Medical College and KEM Hospital Mumbai Maharashtra India

Department of Endocrinology University Medical Center Groningen Groningen Netherlands

Department of Internal Medicine 1 University Hospital P J Šafárik University Košice Slovakia

Department of Internal Medicine Radboud University Medical Center Nijmegen Netherlands

Department of Laboratory Medicine and Pathobiology University of Toronto Toronto Ontario Canada

Department of Medical Genetics University of Cambridge Cambridge Biomedical Campus Cambridge UK

Department of Medical Sciences Uppsala University Uppsala Sweden

Department of Medicine 4 University Hospital Ludwig Maximilians University Munich Munich Germany

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

Department of Medicine St Vincent's Hospital Medical School Melbourne Victoria Australia

Department of Nuclear Medicine Aix Marseille University La Timone University Hospital Marseille France

Department of Nuclear Medicine and Molecular Imaging Institut de Cancérologie de Strasbourg Europe IPHC UMR 7178 CNRS University of Strasbourg Strasbourg France

Department of Oncogenetics and Cancer Genomic Medicine AP HP Hôpital européen Georges Pompidou Paris France

Department of Otolaryngology Head and Neck Surgery Icahn School of Medicine at Mount Sinai New York NY USA

Department of Pathology University Medical Center Utrecht Utrecht Netherlands

Department of Surgery UCSF Mount Zion San Francisco CA USA

Department of Surgical Oncology MD Anderson Cancer Center Houston TX USA

Developmental Therapeutics Branch Center for Cancer Research National Cancer Institute National Institutes of Health Bethesda MD USA

Division of Endocrine Surgery Department of Surgery Mayo Clinic Rochester MN USA

Division of Endocrinology and Metabolism Department of Medicine University of Florida Gainesville FL USA

Division of Endocrinology Department of Medicine and Research Center Centre hospitalier de l'Université de Montréal Montréal Québec Canada

Division of Endocrinology Department of Medicine Centre de recherche du Centre hospitalier de l'Université de Montréal Université de Montréal Montréal Canada

Division of Endocrinology Diabetes Metabolism and Nutrition Mayo Clinic Rochester MN USA

Green Templeton College University of Oxford Oxford UK

Hereditary Endocrine Cancer Group Spanish National Cancer Research Center Madrid Spain

Hypertension Unit Hôpital Européen Georges Pompidou Assistance Publique Hôpitaux de Paris Paris France

Institute of Clinical Chemistry and Laboratory Medicine University Hospital Carl Gustav Carus at the TU Dresden Dresden Germany

James J Peters VA Medical Center New York City NY USA

Malcom Randall VA Medical Center Gainesville FL USA

Molecular Imaging and Therapy Service Radiology Department Memorial Sloan Kettering Cancer Center New York NY USA

Molecular Imaging Program National Cancer Institute National Institutes of Health Bethesda MD USA

NET Unit Royal Free Hospital London UK

NSW Health Pathology Department of Anatomical Pathology Royal North Shore Hospital St Leonards New South Wales Australia

Princess Máxima Center for paediatric oncology Utrecht Netherlands

Research Center CHU Sainte Justine and Dept of Paediatrics University of Montreal Montreal Québec Canada

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

Sorbonne University Department of Nuclear Medicine Pitié Salpêtrière Paris France

Stanford University School of Medicine Department of Physician Assistant Studies Stanford CA USA

Surgical Oncology Program National Cancer Institute National Institutes of Health Bethesda MD USA

Université Paris Cité Inserm PARCC Equipe Labellisée par la Ligue contre le Cancer Paris France

University of Sydney Sydney NSW Australia Cancer Diagnosis and Pathology Group Kolling Institute of Medical Research Royal North Shore Hospital St Leonards New South Wales Australia

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$a Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement / $c D. Taïeb, S. Nölting, ND. Perrier, M. Fassnacht, JA. Carrasquillo, AB. Grossman, R. Clifton-Bligh, GB. Wanna, ZG. Schwam, L. Amar, I. Bourdeau, RT. Casey, J. Crona, CL. Deal, J. Del Rivero, QY. Duh, G. Eisenhofer, T. Fojo, HK. Ghayee, AP. Gimenez-Roqueplo, AJ. Gill, R. Hicks, A. Imperiale, A. Jha, MN. Kerstens, RR. de Krijger, A. Lacroix, I. Lazurova, FI. Lin, C. Lussey-Lepoutre, ER. Maher, O. Mete, M. Naruse, N. Nilubol, M. Robledo, F. Sebag, NS. Shah, A. Tanabe, GB. Thompson, HJLM. Timmers, J. Widimsky, WJ. Young, L. Meuter, JWM. Lenders, K. Pacak

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$a Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

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