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E Kim Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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E M Rath Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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V H M Tsang Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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A P Duff Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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B G Robinson Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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W B Church Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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D E Benn Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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T Dwight Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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R J Clifton-Bligh Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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context of inherited neoplasia syndromes in more than 30% of cases and are linked to germline mutations in succinate dehydrogenase subunit A ( SDHA ), SDHB , SDHC , SDHD , SDHAF2 , VHL , RET , NF1 , TMEM127 , MAX , KIF1B , EPAS1 ( Lorenzo et

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Maya B Lodish
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Karen T Adams Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Thanh T Huynh Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Tamara Prodanov Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Alex Ling Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Clara Chen Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Suzanne Shusterman Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Camilo Jimenez Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Maria Merino Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Marybeth Hughes Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Kendall W Cradic Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Dragana Milosevic Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Ravinder J Singh Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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Constantine A Stratakis
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Karel Pacak Section on Endocrinology and Genetics and Pediatric Endocrinology Program, Reproductive Biology and Adult Endocrinology Program, Department of Diagnostic Radiology, Department of Pediatric Oncology, The University of Texas M.D. Anderson Cancer Center, Pathology, Surgery, Department of Laboratory Medicine and Pathology, Program on Developmental Endocrinology and Genetics (PDEGEN), National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, 10 Center Drive, Room 1-3330, Bethesda, Maryland 20892, USA

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been reported in the literature, but their association with specific mutations and/or genetic syndromes has not been described ( Subramanian & Maker 2006 ). Germline mutations in the genes encoding subunits B, C, and D of succinate dehydrogenase (SDH

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Hans K Ghayee
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Bas Havekes Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Eleonora P M Corssmit Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Graeme Eisenhofer Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Stephen R Hammes
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Zahid Ahmad
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Alexander Tessnow
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Ivica Lazúrová Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Karen T Adams Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Antonio T Fojo Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Karel Pacak Division of Endocrinology, Reproductive Biology and Medicine Program, Department of Endocrinology and Metabolism, Institute of Clinical Chemistry & Laboratory Medicine and Department of Medicine, Department of Medicine, Medical Oncology Branch, Department of Internal Medicine, Southwesterm Medical Center, University of Texas, 5323 Harry Hines Boulevard, Suite Y-5.318, Dallas, Texas 75390-8857, USA

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Richard J Auchus
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2, neurofibromatosis type 1, and von Hippel–Lindau syndrome are familial syndromes that predominantly predispose to adrenal pheochromocytomas. By contrast, mutations in the genes encoding succinate dehydrogenase (SDH) subunits B, C, and D cause extra

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E Rapizzi Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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T Ercolino Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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L Canu Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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V Giaché Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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M Francalanci Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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C Pratesi Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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A Valeri Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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M Mannelli Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy
Endocrinology Unit, Endocrinology Unit, Vascular Surgery Unit, Chirurgia Generale, Istituto Toscano Tumori, Department of Clinical Pathophysiology, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy

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includes the genes encoding the four subunits of the succinate dehydrogenase (SDH), enzyme involved in the tricarboxylic acid cycle ( Baysal et al . 2000 , Niemann & Müller 2000 , Astuti et al . 2001 , Burnichon et al . 2010 ) and in the mitochondrial

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Francesca Amore Department Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy

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Rachele Garella Department Experimental and Clinical Medicine, University of Florence, Florence, Italy

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Alice Santi Department Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy

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Daniele Guasti Department of Experimental and Clinical Medicine, Imaging Platform, University of Florence, Italy

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Serena Martinelli Department Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, Florence, Italy
ENS@T Center of Excellence, Florence, Italy

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Letizia Canu Department Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, Florence, Italy
ENS@T Center of Excellence, Florence, Italy

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Daniele Bani Department of Experimental and Clinical Medicine, Imaging Platform, University of Florence, Italy

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Jiri Neuzil Institute of Biotechnology, Czech Academy of Sciences, Prague-West, Czech Republic
School of Pharmacy and Medical Science, Griffith University, Southport, Queensland, Australia
Faculty of Science and 1st Medical Faculty, Charles University, Prague, Czech Republic

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Mario Maggi Department Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, Florence, Italy
ENS@T Center of Excellence, Florence, Italy

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Roberta Squecco Department Experimental and Clinical Medicine, University of Florence, Florence, Italy

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Elena Rapizzi Department Experimental and Clinical Medicine, University of Florence, Florence, Italy
Centro di Ricerca e Innovazione sulle Patologie Surrenaliche, AOU Careggi, Florence, Italy
ENS@T Center of Excellence, Florence, Italy

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al. 2021 , Jhawar et al. 2022 ). These genes include those encoding the four subunits of succinate dehydrogenase (SDH) (SDH subunit A (SDHA), SDH subunit B (SDHB), SDH subunit C (SDHC) and SDH subunit D (SDHD)). PPGLs are predominantly non

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Paraskevi Xekouki Section on Endocrinology and Genetics (SEGEN), Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Inter-Institute Pediatric Endocrinology Program, National Institutes of Health (NIH), Building 10, CRC, Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Constantine A Stratakis Section on Endocrinology and Genetics (SEGEN), Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Inter-Institute Pediatric Endocrinology Program, National Institutes of Health (NIH), Building 10, CRC, Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Introduction Succinate dehydrogenase (SDH) or succinate-coenzyme Q reductase is a multimeric enzyme that is bound to the inner membrane of mitochondria ( Oyedotun & Lemire 2004 ). It has a dual role as it serves both as a critical step of the

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Tomáš Zelinka Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development
Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Henri J L M Timmers Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Anna Kozupa Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Clara C Chen Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Jorge A Carrasquillo Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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James C Reynolds Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Alexander Ling Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Graeme Eisenhofer Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Ivica Lazúrová Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Karen T Adams Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Millie A Whatley Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Jiří Widimský Jr Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Karel Pacak Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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mitochondrial complex II enzyme succinate dehydrogenase enzyme subunit B ( SDHB ; Amar et al . 2005 , Benn et al . 2006 , Brouwers et al . 2006 , Timmers et al . 2007 ). The main sites of metastatic spread of malignant PHEO/PGL are lymphatic nodes (local

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Paul Benjamin Loughrey Patrick G Johnston Centre for Cancer Research, Queen’s University, Belfast, UK
Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Federico Roncaroli Geoffrey Jefferson Brain Research Centre, Division of Neuroscience and Experimental Psychology, School of Medicine, Manchester University, Manchester, UK

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Estelle Healy Department of Cellular Pathology, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Philip Weir Department of Neurosurgery, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Madhu Basetti Cancer Research UK Cambridge Institute, Li Ka Shing Centre, University of Cambridge, Cambridge, UK

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Ruth T Casey Department of Endocrinology, Cambridge University Hospital NHS Foundation Trust, Cambridge, UK

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Steven J Hunter Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Márta Korbonits Centre for Endocrinology, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK

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coincidental. Succinate dehydrogenase (SDH) gene variants (collectively known as SDHx ) can associate with PPGL ( Baysal et al. 2000 ). The association of PitNET and PPGL in the setting of SDHx variant was established at the molecular level in 2012

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William Beimers Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Megan Braun Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Kaleb Schwinefus Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Keenan Pearson Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Brandon Wilbanks Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Louis James Maher Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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surgery. About 25% of PGL cases are hereditary, and most hereditary PGLs are linked to pathogenic variants in nuclear genes encoding the four subunits of the tricarboxylic acid (TCA) cycle enzyme succinate dehydrogenase (SDH; also complex II of the

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Adrian F Daly Department of Endocrinology Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart‐Tilman, 4000 Liège, Belgium

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Albert Beckers Department of Endocrinology Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart‐Tilman, 4000 Liège, Belgium

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& Carney 2009 ). Succinate dehydrogenase gene dysregulation is instrumental in orchestrating various endocrine cancer syndromes The past few decades have observed remarkable successes in determining the causes of paragangliomas and GIST, two of the

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