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. Gimenez-Roqueplo AP , Favier J, Rustin P, Rieubland 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
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implications ( Rindi et al . 2000 , Turner et al . 2006 , Vinik et al . 2009 ). Neuroendocrine cells can be confined to a specific gland, such as the adrenal medulla (phaeochromocytomas) or the thyroid (medullary thyroid carcinoma). These tumours will not
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.1186/1471-2350-10-34 ) 19368708 10.1186/1471-2350-10-34 Bayley JP Kunst HP Cascon A Sampietro ML Gaal J Korpershoek E Hinojar-Gutierrez A Timmers HJ Hoefsloot LH Hermsen MA , et al . 2010 SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma . Lancet
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Department of Pathology and Laboratory Medicine, Department of Pathology, Department of Pathology, Tufts Medical Center, Tufts University School of Medicine, 800 Washington Street, Box 802, Boston, Massachusetts 02111, USA
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) , and the Grading system for Adrenal Phaeochromocytoma and Paraganglioma (GAPP), proposed by Kimura et al . (2014) ( Fig. 4 ). While PASS applies only to pheochromocytomas, GAPP applies to both pheochromocytomas and paragangliomas. Morphological
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-sensing defect . Human Genetics 113 228 – 237 . ( doi:10.1007/s00439-003-0969-6 ). Astuti D Douglas F Lennard TW Aligianis IA Woodward ER Evans DG Eng C Latif F Maher ER 2001a Germline SDHD mutation in familial phaeochromocytoma
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Hereditary phaeochromocytoma and paragangliomas: a study of five susceptibility genes. Journal of Medical Genetics 40 e75 . Benn DE , Dwight T, Richardson AL, Delbridge L, Bambach CP, Stowasser M, Gordon RD, Marsh
Barts and the London School of Medicine, Department of Endocrinology and Internal Medicine, Centre for Endocrinology, London EC1M 6BQ, UK
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-entero-pancreatic and bronchial NETs and phaeochromocytomas and paragangliomas. They may occur sporadically or in a familial context of autosomal dominant inherited syndromes such as multiple endocrine neoplasia (MEN1 and MEN2), VHL, Carney complex (principally
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their journey in hopes of advancing awareness and research. References Andersen GS Toftdahl DB Lund JO Strandgaard S & Nielsen PE 1988 The incidence rate of phaeochromocytoma and Conn’s syndrome in Denmark, 1977–1981 . Journal of
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Department of Internal Medicine and Oncology, Faculty of Medicine, Semmelweis University, Budapest, Hungary
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.1016/j.ijsu.2022.106853 ) Lenders JWM Kerstens MN Amar L Prejbisz A Robledo M Taieb D Pacak K Crona J Zelinka T Mannelli M , et al. 2020 Genetics, diagnosis, management and future directions of research of phaeochromocytoma and
Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Madrid, Spain
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had diabetes mellitus before surgery, with complete resolution in 79% ( n = 33) after extirpation of the phaeochromocytoma. Similarly, Liu et al . (2019) described a remission rate of diabetes of 70%, with high BMI as a risk factor for persistent