title: 15 YEARS OF PARAGANGLIOMA: Metabolism and pheochromocytoma/paraganglioma.
abstract: The discovery of SDHD as a pheochromocytoma/paraganglioma susceptibility gene was the prismatic event that led to all of the subsequent work highlighting the key roles played by mitochondria in the pathogenesis of these tumors and other solid cancers. Alterations in the function of tricarboxylic acid cycle enzymes can cause accumulation of intermediate substrates and subsequent changes in cell metabolism, activation of the angiogenic pathway, increased reactive oxygen species production, DNA hypermethylation, and modification of the tumor microenvironment favoring tumor growth and aggressiveness. The elucidation of these tumorigenic mechanisms should lead to novel therapeutic targets for the treatment of the most aggressive forms of pheochromocytoma/paraganglioma.
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA massimo.mannelli@unifi.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Mannelli
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Elena
authors.initials: E
authors.lastname: Rapizzi
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Rossella
authors.initials: R
authors.lastname: Fucci
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Letizia
authors.initials: L
authors.lastname: Canu
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Tonino
authors.initials: T
authors.lastname: Ercolino
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Michaela
authors.initials: M
authors.lastname: Luconi
authors.affiliation: Endocrinology UnitDepartment of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Florence, Viale Pieraccini 6, 50139 Florence, ItalyEndocrinology UnitCareggi Hospital, Azienda Ospedaliera Universitaria Careggi, Florence, ItalyDivision of EndocrinologyDiabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: William F
authors.initials: WF
authors.lastname: Young
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000378:metabolism*; D019175:DNA Methylation; D006801:Humans; D000860:Hypoxia / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism; D010235:Paraganglioma / Q000378:metabolism*; D010673:Pheochromocytoma / Q000378:metabolism*; D017382:Reactive Oxygen Species; D013385:Succinate Dehydrogenase / Q000172:deficiency
chemical_list: D017382:Reactive Oxygen Species; D013385:Succinate Dehydrogenase
keywords: angiogenesis; mitochondria; oncometabolites; pheochromocytoma/paraganglioma; succinatedehydrogenase