Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a young age or late in the disease course, which presents challenges in balancing surgical intervention with various medical and radiotherapeutic approaches. The axiom-first, do no harm-should always be considered and an initial period of observation (ie, watchful waiting) is often appropriate to characterise tumour behaviour in patients with these pathogenic variants. These patients should be referred to specialised high-volume medical centres. This consensus guideline aims to help physicians with the clinical decision-making process when caring for patients with SDHD PPGLs.

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

Department of Cardiothoracic and Vascular Surgery UTHealth Houston McGovern Medical School Memorial Hermann Hospital Heart and Vascular Institute Houston TX USA

Department of Endocrine Surgery Aix Marseille University Conception University Hospital Marseille France

Department of Endocrinology Aix Marseille University Conception University Hospital Marseille France; INSERM U1251 Aix Marseille University Conception University Hospital Marseille France

Department of Endocrinology Center of Endocrine Tumors Leiden Leiden University Medical Centre Leiden Netherlands

Department of Endocrinology Royal North Shore Hospital Sydney NSW Australia; Cancer Genetics Laboratory Kolling Institute University of Sydney Sydney NSW Australia

Department of Endocrinology University Medical Center Groningen Groningen Netherlands

Department of Internal Medicine Radboud University Medical Center Nijmegen Netherlands

Department of Laboratory Medicine and Pathobiology University of Toronto Toronto ON Canada; Endocrine Pathology Society Toronto ON Canada

Department of Medical Genetics NIHR Cambridge Biomedical Research Centre Cancer Research UK Cambridge Centre Cambridge Biomedical Campus University of Cambridge Cambridge UK

Department of Medicine ΙΙI University Hospital Carl Gustav Carus Technische Universität Dresden Dresden Germany; Department of Internal Medicine Radboud University Medical Center Nijmegen Netherlands

Department of Neurosurgery Stanford University School of Medicine Palo Alto CA USA

Department of Neurosurgical Surgery Rutgers New Jersey Medical School Newark NJ USA

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 Otolaryngology and Head and Neck Surgery Dutch Academic Alliance Skull Base Pathology Radboud University Medical Center Nijmegen Netherlands; Department of Otolaryngology and Head and Neck Surgery Dutch Academic Alliance Skull Base Pathology Maastricht University Medical Center Maastricht Netherlands

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

Department of Otorhinolaryngology and Head and Neck Surgery University Hospital of Verona Verona Italy

Department of Otorhinolaryngology Leiden University Medical Centre Leiden Netherlands

Department of Pathology University Medical Center Utrecht Utrecht Netherlands; Princess Máxima Center for Pediatric Oncology Utrecht Netherlands

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

Department of Radiation Oncology Stanford University School of Medicine Palo Alto CA USA

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

Department of Surgical Oncology MD Anderson Cancer Center Houston TX USA

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

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

Division of Endocrinology and Metabolism Department of Medicine Malcom Randall VA Medical Center University of Florida Gainesville FL USA

Division of Endocrinology Department of Medicine and Research Center Centre Hospitalier de l'Université de Montréal Montréal QC Canada

Green Templeton College University of Oxford Oxford UK; NET Unit Royal Free Hospital London UK

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

Medical Center and Endocrine Center Ijinkai Takeda General Hospital Kyoto Japan

Section of Endocrinology and Metabolism Department of Medicine Faculty of Medicine and Surgery University of Santo Tomas Hospital University of Santo Tomas Manila Philippines

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

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

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

Université Paris Cité Inserm PARCC Equipe Labellisée par la Ligue contre le Cancer Paris France; Département de Médecine Génomique des Tumeurs et des Cancers Hôpital Européen Georges Pompidou Assistance Publique Hôpitaux de Paris Paris France

Université Paris Cité Inserm PARCC Equipe Labellisée par la Ligue contre le Cancer Paris France; Department of Nuclear Medicine Pitié Salpêtrière Hospital Sorbonne University Paris France

Université Paris Cité Inserm PARCC Equipe Labellisée par la Ligue contre le Cancer Paris France; Unité d'hypertension artérielle Hôpital Européen Georges Pompidou Assistance Publique Hôpitaux de Paris Paris France

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Should Methoxytyramine Routinely Be Included in the Guidelines for Biochemical Assessment of Pheochromocytomas and Paragangliomas?

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

High incidence of occult familial SDHD cases amongst Czech patients with head and neck paragangliomas