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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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. Journal of the American Medical Association 244 1236 – 1237 . ( doi:10.1001/jama.1980.03310110046029 ). Anderson RJ Lufkin EG Sizemore GW Carney JA Sheps SG Silliman YE 1981 Acromegaly and pituitary adenoma with phaeochromocytoma: a

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Noriko Kimura Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Kazuhiro Takekoshi Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Akira Horii Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Ryo Morimoto Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tsuneo Imai Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Yutaka Oki Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tomohito Saito Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Sanae Midorikawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tadashi Arao Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Chiho Sugisawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Masanobu Yamada Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Yuichi Otuka Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Isao Kurihara Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Kokichi Sugano Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Minoru Nakane Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Atsushi Fukuuchi Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Takumi Kitamoto Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Jun Saito Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tetsuo Nishikawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Mitsuhide Naruse Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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C Crespin M Nau V Khau Van Kien P Corvol P Plouin PF Jeunemaitre X 2003 Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas . Cancer Research 63 5615 – 5621 . Kimura N Takayanagi

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Hartmut P H Neumann Unit for Preventive Medicine, Department of Internal Medicine, University Medical Center, Albert‐Ludwigs‐University, Hugstetter Straße 55, D‐79106 Freiburg, Germany

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Wouter de Herder Unit for Preventive Medicine, Department of Internal Medicine, University Medical Center, Albert‐Ludwigs‐University, Hugstetter Straße 55, D‐79106 Freiburg, Germany

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.1086/321282 ). Bayley JP Kunst HP Cascon A Sampietro ML Gaal J Korpershoek E Hinojar-Gutierrez A Timmers HJ Hoefsloot LH Hermsen MA 2010 SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma . Lancet. Oncology 11

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Ruth T Casey Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre and Cancer Research UK Cambridge Centre, Cambridge, UK
Department of Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK

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Benjamin G Challis Department of Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK

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Alison Marker Department of Histopathology, Cambridge University NHS Foundation Trust, Cambridge, UK

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Deborah Pitfield Department of Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK

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Heok K Cheow Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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Ashley Shaw Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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Soo-Mi Park Department of Medical Genetics, University of Cambridge and NIHR Cambridge Biomedical Research Centre and Cancer Research UK Cambridge Centre, Cambridge, UK

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the development of phaeochromocytoma (PC) and PGL tumours over 15 years ago, SDHA was first associated with PGL only seven years ago ( Burnichon et al . 2010 ) and, more recently, with wild-type gastrointestinal stromal tumours (WT GIST) ( Evenepoel

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Department of Medical Genetics, Department of Clinical Genetics, University of Cambridge, Cambridge, UK and

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Gentle D Martinsson T Kogner P Neumann HP Voutilainen R Eng C 2004 Investigation of the role of SDHB inactivation in sporadic phaeochromocytoma and neuroblastoma . British Journal of Cancer 91 1835 – 1841 . ( doi:10.1038/sj

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score of 1 or 2. In only one out of six cases scoring 2 did one physician score the mass 2 while the other scored it 3. Discussion CT or MRI represent the first diagnostic step in the localization of phaeochromocytoma ( Van Gils et

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(s)/paraganglioma(s) . World Journal of Surgery 14 325 – 329 . van der Horst-Schrivers AN Kerstens MN Wolffenbuttel BH 2006 Preoperative pharmacological management of phaeochromocytoma . Netherlands Journal of Medicine 64 290 – 295 . Ilias I Shulkin B

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Anne-Paule Gimenez-Roqueplo Department of Endocrinology, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, INSERM, Endocrinology Unit, Reproductive and Adult Endocrinology Program, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
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Massimo Mannelli Department of Endocrinology, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, INSERM, Endocrinology Unit, Reproductive and Adult Endocrinology Program, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands

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Karel Pacak Department of Endocrinology, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, INSERM, Endocrinology Unit, Reproductive and Adult Endocrinology Program, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands

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. 2003 ). Also there is a tendency for SDHD mutation carriers of truncating mutations to develop phaeochromocytoma in addition to HNPGL ( Astrom et al . 2003 , Benn et al . 2006 ). Biochemical diagnosis and imaging Suspicion of sPGL is usually

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Clinical Genomics Linköping, Linköping University, Linköping, Sweden

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genetics of phaeochromocytoma and paraganglioma . Archives of Endocrinology and Metabolism 61 490 – 500 . ( https://doi.org/10.1590/2359-3997000000299 ) Kenny TC Hart P Ragazzi M Sersinghe M Chipuk J Sagar MAK Eliceiri KW LaFramboise T

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Patricia Compagnon INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Erwan Thouennon INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Christian Bastard INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Isabelle Lihrmann INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Perrine Pichon INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Christian Thuillez INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Youssef Anouar INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Jean-Marc Kuhn INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Laurent Yon INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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Hervé Lefebvre INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France
INSERM U413, INSERM U644, Laboratory of Oncologic Genetics, Department of Endocrinology, Hypertension Unit, INSERM U567, EA4310, DC2N, IFRMP 23, University of Rouen, Mont Saint Aignan, France

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induced by metoclopramide in patient with pheochromocytoma . Angiology 35 122 – 128 . Adler-Graschinsky E Rubio MC Barontini De Moyano M 1984 Metoclopramide increases the release of catecholamines from isolated human phaeochromocytomas

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