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Pheochromocytomas in Complex Genetic Disorders

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Polyendocrine Disorders and Endocrine Neoplastic Syndromes

Part of the book series: Endocrinology ((ENDOCR))

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Abstract

Pheochromocytoma and paraganglioma are tumors of the sympathetic or parasympathetic paraganglia. When the paraganglioma arises from the adrenal medulla, which is of the main sympathetic paraganglia, it is called pheochromocytoma. Both of them originate from neural crest cells and share similar if not identical mechanism of tumor development. The same genetic alteration may predispose to the development of sympathetic and parasympathetic paraganglioma.

The best known hereditary forms of pheochromocytoma and paraganglioma are the von Hippel-Lindau disease, in which pheochromocytoma may be associated with hemangioblastomas of the central nervous system, retinal angiomas, pancreatic endocrine tumors/cysts, and renal clear cell carcinomas/cysts; the multiple endocrine neoplasia type 2, in which pheochromocytoma is associated with medullary thyroid carcinoma and primary hyperparathyroidism; and the type 1 neurofibromatosis, the most frequent hereditary cancer syndrome. A relatively new entity is the paraganglioma syndrome, in which sympathetic and parasympathetic paragangliomas are variously associated and mainly caused by mutations of one of the genes coding for the succinate dehydrogenases complex units. Additionally, familial non-syndromic pheochromocytomas may be caused by mutations of TMEM127 and MAX genes.

The list of predisposing gene is quite long and comprises other genes that may predispose to pheochromocytoma/paraganglioma development. Considering the progress of molecular biology and translational science, it is likely that this list is not yet completed.

Dr. Opocher and Dr. Ferrara have equally contributed

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References

  • Amar L, Bertherat J, Baudin E, Ajzenberg C, Bressac-de Paillerets B, Chabre O, Chamontin B, Delemer B, Giraud S, Murat A, Niccoli-Sire P, Richard S, Rohmer V, Sadoul JL, Strompf L, Schlumberger M, Bertagna X, Plouin PF, Jeunemaitre X, Gimenez-Roqueplo AP. Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol. 2008;23:8812–8.

    Article  Google Scholar 

  • Arstrom K, Cohen JE, Willet-Brozick C, Aston E, Baysal B. Altitude is a phenotypic modifier in hereditary paraganglioma type 1: evidence for an oxygen-sensing defect. Hum Genet. 2003;113:228–37.

    Article  Google Scholar 

  • Astuti D, Douglas F, Lennard TWJ, Agaliaganis I, Woodward ER, Evans DGR, Eng C, Latif F, Maher ER. Germline SDHD mutation in familial phaeochromocytoma. Lancet. 2001a;357:1181–2.

    Article  CAS  Google Scholar 

  • Astuti D, Latif F, Dallol A, Dahia PLM, Douglas F, George E, Sköldberg F, Husebye ES, Eng C, Maher ER. Mutations in the mitochondrial complex II subunit SDHB cause susceptibility to familial paraganglioma and pheochromocytoma. Am J Hum Genet. 2001b;69:49–54.

    Article  CAS  Google Scholar 

  • Bausch B, Borozdin W, Mautner VF, Hoffmann MM, Boehm D, Robledo M, Cascon A, Harenberg T, Schiavi F, Pawlu C, Peczkowska M, Letizia C, Calvieri S, Arnaldi G, Klingenberg-Noftz RD, Reisch N, Fassina A, Brunaud L, Walter MA, Mannelli M, MacGregor G, Palazzo FF, Barontini M, Walz MK, Kremens B, Brabant G, Pfäffle R, Koschker AC, Lohoefner F, Mohaupt M, Gimm O, Jarzab B, McWhinney SR, Opocher G, Januszewicz A, Kohlhase J, Eng C, Neumann HP, European-American Phaeochromocytoma Registry Study Group. Germline NF1 mutational spectra and loss-of-heterozygosity analyses in patients with pheochromocytoma and neurofibromatosis type 1. J Clin Endocrinol Metab. 2007;92(7):2784–92.

    Article  CAS  Google Scholar 

  • Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH, European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group. Clinical characterization of the pheochromocytoma and paraganglioma susceptibility genes SDHA, TMEM127, MAX, and SDHAF2 for gene-informed prevention. JAMA Oncol. 2017;3:1204–12.

    Article  Google Scholar 

  • Baysal BE. Hereditary paraganglioma targets diverse paraganglia. J Med Genet. 2002;39(9):617–22.

    Article  CAS  Google Scholar 

  • Baysal BE, Ferrell RE, Willett-Brozick JE, Lawrence EC, Myssiorek D, Bosch A, van der Mey A, Taschner PE, Rubinstein WS, Myers EN, Richard CW 3rd, Cornelisse CJ, Devilee P, Devlin B. Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science. 2000;287(5454):848–51.

    Article  CAS  Google Scholar 

  • Baysal BE, Willett-Brozick JE, Filho PA, Lawrence EC, Myers EN, Ferrell RE. An Alu-mediated partial SDHC deletion causes familial and sporadic paraganglioma. J Med Genet. 2004;41(9):703–9.

    Article  CAS  Google Scholar 

  • Benn DE, Gimenez-Roqueplo AP, Reilly JR, Bertherat J, Burgess J, Byth K, Croxson M, Dahia PL, Elston M, Gimm O, Henley D, Herman P, Murday V, Niccoli-Sire P, Pasieka JL, Rohmer V, Tucker K, Jeunemaitre X, Marsh DJ, Plouin PF, Robinson BG. Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes. J Clin Endocrinol Metab. 2006;91(3):827–36.

    Article  CAS  Google Scholar 

  • Bravo EL, Gifford RW Jr. Current concepts. Pheochromocytoma: diagnosis, localization and management. N Engl J Med. 1984;311(20):1298–303.

    Article  CAS  Google Scholar 

  • Burnichon N, Rohmer V, Amar L, Herman P, Leboulleux S, Darrouzet V, Niccoli P, Gaillard D, Chabrier G, Chabolle F, Coupier I, Thieblot P, Lecomte P, Bertherat J, Wion-Barbot N, Murat A, Venisse A, Plouin PF, Jeunemaitre X, Gimenez-Roqueplo AP, PGL.NET network. The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. J Clin Endocrinol Metab. 2009;94(8):2817–27.

    Article  CAS  Google Scholar 

  • Burnichon N, Brière JJ, Libé R, Vescovo L, Rivière J, Tissier F, Jouanno E, Jeunemaitre X, Bénit P, Tzagoloff A, Rustin P, Bertherat J, Favier J, Gimenez-Roqueplo AP. SDHA is a tumor suppressor gene causing paraganglioma. Hum Mol Genet. 2010;19(15):3011–20.

    Article  CAS  Google Scholar 

  • Burnichon N, Mazzella JM, Drui D, Amar L, Bertherat J, Coupier I, Delemer B, Guilhem I, Herman P, Kerlan V, Tabarin A, Wion N, Lahlou-Laforet K, Favier J, Gimenez-Roqueplo AP. Risk assessment of maternally inherited SDHD paraganglioma and phaeochromocytoma. J Med Genet. 2017;54(2):125–33.

    Article  Google Scholar 

  • Casey RT, Ascher DB, Rattenberry E, Izatt L, Andrews KA, Simpson HL, Challis B, Park SM, Bulusu VR, Lalloo F, Pires DEV, West H, Clark GR, Smith PS, Whitworth J, Papathomas TG, Taniere P, Savisaar R, Hurst LD, Woodward ER, Maher ER. SDHA related tumorigenesis: a new case series and literature review for variant interpretation and pathogenicity. Mol Genet Genomic Med. 2017;5:237–50.

    Article  CAS  Google Scholar 

  • Chen F, Kishida T, Yao M, Hustad T, Glavac D, Dean M, Gnarra JR, Orcutt ML, Duh FM, Glenn G, Green J, Hsia YE, Lamiell J, Li H, Wei MH, Schmidt L, Tory K, Kuzman I, Stackhouse T, Latif F, Linehan WM, Lerman M, Zbar B. Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: correlations with phenotype. Hum Mutat. 1995;5:66–75.

    Article  CAS  Google Scholar 

  • Comino-Méndez I, Gracia-Aznárez FJ, Schiavi F, Landa I, Leandro-García LJ, Letón R, Honrado E, Ramos-Medina R, Caronia D, Pita G, Gómez-Graña A, de Cubas AA, Inglada-Pérez L, Maliszewska A, Taschin E, Bobisse S, Pica G, Loli P, Hernández-Lavado R, Díaz JA, Gómez-Morales M, González-Neira A, Roncador G, Rodríguez-Antona C, Benítez J, Mannelli M, Opocher G, Robledo M, Cascón A. Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma. Nat Genet. 2011;43:663–7.

    Article  Google Scholar 

  • Crona J, Taïeb D, Pacak K. New perspectives on pheochromocytoma and paraganglioma: toward a molecular classification. Endocr Rev. 2017;38(6):489–515. https://doi.org/10.1210/er.2017-00062. PubMed PMID: 28938417.

    Article  PubMed  PubMed Central  Google Scholar 

  • Dahia PL, Familial Pheochromocytoma Consortium. Transcription association of VHL and SDH mutations link hypoxia and oxidoreductase signals in pheochromocytomas. Ann N Y Acad Sci. 2006;1073:208–20.

    Article  CAS  Google Scholar 

  • Eisenhofer G, Huynh TT, Pacak K, Brouwers FM, Walther MM, Linehan WM, Munson PJ, Mannelli M, Goldstein DS, Elkahloun AG. Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia-driven angiogenic pathways in von Hippel-Lindau syndrome. Endocr Relat Cancer. 2004;11(4):897–911.

    Article  CAS  Google Scholar 

  • Eisenhofer G, Klink B, Richter S, Lenders JW, Robledo M. Metabologenomics of phaeochromocytoma and paraganglioma: an integrated approach for personalised biochemical and genetic testing. Clin Biochem Rev. 2017;38(2):69–100.

    PubMed  PubMed Central  Google Scholar 

  • Elbehti-Green A, Au HC, Mascarello JT, Ream-Robinson D, Scheffler IE. Characterization of the human SDHC gene encoding of the integral membrane proteins of succinate-quinone oxidoreductase in mitochondria. Gene. 1998;213(1–2):133–40.

    Article  CAS  Google Scholar 

  • Fränkel F. Ein Fall von doppelseitigem, völlig latent verlaufenen Nebennierentumor und gleichzeitiger Nephritis mit Veränderungen am Circulationsapparat und Retinitis. Arch Pathol Anat Physiol Klin Med. 1886;103:244–63.

    Article  Google Scholar 

  • Gaal J, Burnichon N, Korpershoek E, Roncelin I, Bertherat J, Plouin PF, de Krijger RR, Gimenez-Roqueplo AP, Dinjens WN. Isocitrate dehydrogenase mutations are rare in pheochromocytomas and paragangliomas. J Clin Endocrinol Metab. 2010;95(3):1274–8.

    Article  CAS  Google Scholar 

  • Gimenez-Roqueplo AP, Favier J, Rustin P, Mourad JJ, Plouin PF, Corvol P, Rotig A, Jeunemaitre X. The R22X mutation of the SDHD gene in hereditary paraganglioma abolishes the enzymatic activity of complex II in the mitochondrial respiratory chain and activates the hypoxia pathway. Am J Hum Genet. 2001;69(6):1186–97.

    Article  CAS  Google Scholar 

  • Gimm O, Koch CA, Januszewicz A, Opocher G, Neumann HP. The genetic basis of pheochromocytoma. Front Horm Res. 2004;31:45–60.

    Article  CAS  Google Scholar 

  • Hao HX, Khalimonchuk O, Schraders M, Dephoure N, Bayley JP, Kunst H, Devilee P, Cremers CW, Schiffman JD, Bentz BG, Gygi SP, Winge DR, Kremer H, Rutter J. SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science. 2009;325(5944):1139–42.

    Article  CAS  Google Scholar 

  • Hes FJ, Hoppener JW, Lips CJ. Clinical review 155: pheochromocytoma in Von Hippel-Lindau disease. J Clin Endocrinol Metab. 2003;88(3):969–74.

    Article  CAS  Google Scholar 

  • Kaelin WG Jr. The VHL tumor suppressor gene: insights into oxygen sensing and cancer. Trans Am Clin Climatol Assoc. 2017;128:298–307.

    PubMed  PubMed Central  Google Scholar 

  • Lack EE. Anatomy and physiology of peripheral arterial chemoreceptors. In: Lack EE, editor. Pathology of adrenal and extra-adrenal paraganglia. Philadelphia: W.B. Saunders; 1994. p. 1–14.

    Google Scholar 

  • Ladroue C, Carcenac R, Leporrier M, Gad S, Le Hello C, Galateau-Salle F, Feunteun J, Pouysségur J, Richard S, Gardie B. PHD2 mutation and congenital erythrocytosis with paraganglioma. N Engl J Med. 2008;359(25):2685–92.

    Article  CAS  Google Scholar 

  • Luis Jaime Castro-Vega, Alexandre Buffet, Aguirre A. De Cubas, Alberto Cascón, Mélanie Menara, Emmanuel Khalifa, Laurence Amar, Sharona Azriel, Isabelle Bourdeau, Olivier Chabre, Maria Currás-Freixes, Valérie Franco-Vidal, Marine Guillaud-Bataille, Christophe Simian, Aurélie Morin, Rocío Letón, Álvaro Gómez-Graña, Patrick J. Pollard, Pierre Rustin, Mercedes Robledo, Judith Favier, Anne-Paule Gimenez-Roqueplo, Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas. Human Molecular Genetics 2014;23(9):2440–2446. https://doi.org/10.1093/hmg/ddt639

  • Neumann HPH, Bausch B, Mcwhinney SR, Bender BU, Gimm O, Franke G, Schipper J, Klisch J, Altehoefer C, Zerres K, Januszewicz A, Eng C. Germ-line mutations in non syndromic pheochromocytoma. N Engl J Med. 2002;346:1459–66.

    Article  CAS  Google Scholar 

  • Neumann HPH, Pawlu C, Peczkowska M, Bausch B, McWhinney SR, Muresan M, Buchta M, Franke G, Klisch J, Bley TA, Hoegerle S, Boedeker CC, Opocher G, Schipper J, Januszewicz A, Eng C. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA. 2004;292:943–51.

    Article  CAS  Google Scholar 

  • Neumann HP, Vortmeyer A, Schmidt D, Werner M, Erlic Z, Cascon A, Bausch B, Januszewicz A, Eng C. Evidence of MEN-2 in the original description of classic pheochromocytoma. N Engl J Med. 2007;357(13):1311–5.

    Article  CAS  Google Scholar 

  • Neumann HPH, Young W, Krauss T, Bayley JP, Schiavi F, Opocher G, Boedeker C, Tirosh A, Castinetti F, Ruf J, Beltsevich D, Walz MK, Groeben H, Von Dobschuetz E, Gimm O, Wohllk N, Pfeifer M, Lourenço DM, Peczkowska M, Patocs A, Ngeow J, Makay O, Shah N, Tischler AS, Leijon H, Pennelli G, Villar Gómez de Las Heras K, Links TP, Bausch B, Eng C. Genetics informs precision practice in the diagnosis and management of pheochromocytoma. Endocr Relat Cancer. 2018. https://doi.org/10.1530/ERC-18-0085. pii: ERC-18-0085. [Epub ahead of print].

  • Niemann S, Muller U. Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Nat Genet. 2000;26(3):268–70.

    Article  CAS  Google Scholar 

  • O’Toole SM, Dénes J, Robledo M, Stratakis CA, Korbonits M. 15 YEARS OF PARAGANGLIOMA: the association of pituitary adenomas and pheochromocytomas or paragangliomas. Endocr Relat Cancer. 2015;22(4):T105–22.

    Article  Google Scholar 

  • Opocher G, Schiavi F, Iacobone M, Toniato A, Sattarova S, Erlic Z, Martella M, Mian C, Merante Boschin I, Zambonin L, De Lazzari P, Murgia A, Pelizzo MR, Favia G, Mantero F. Familial nonsyndromic pheochromocytoma. Ann N Y Acad Sci. 2006;1073:149–55.

    Article  Google Scholar 

  • Pasini B, McWhinney SR, Bei T, Matyakhina L, Stergiopoulos S, Muchow M, Boikos SA, Ferrando B, Pacak K, Assie G, Baudin E, Chompret A, Ellison JW, Briere JJ, Rustin P, Gimenez-Roqueplo AP, Eng C, Carney JA, Stratakis CA. Clinical and molecular genetics of patients with the Carney-Stratakis syndrome and germline mutations of the genes coding for the succinate dehydrogenase subunits SDHB, SDHC, and SDHD. Eur J Hum Genet. 2008;16:79–88.

    Article  CAS  Google Scholar 

  • Qin Y, Yao L, King EE, Buddavarapu K, Lenci RE, Chocron ES, Lechleiter JD, Sass M, Aronin N, Schiavi F, Boaretto F, Opocher G, Toledo RA, Toledo SP, Stiles C, Aguiar RC, Dahia PL. Germline mutations in TMEM127 confer susceptibility to pheochromocytoma. Nat Genet. 2010;42:229–33.

    Article  CAS  Google Scholar 

  • Remacha L, Comino-Méndez I, Richter S, Contreras L, Currás-Freixes M, Pita G, Letón R, Galarreta A, Torres-Pérez R, Honrado E, Jiménez S, Maestre L, Moran S, Esteller M, Satrústegui J, Eisenhofer G, Robledo M, Cascón A. Targeted exome sequencing of Krebs cycle genes reveals candidate cancer-predisposing mutations in pheochromocytomas and paragangliomas. Clin Cancer Res. 2017;23(20):6315–24.

    Article  CAS  Google Scholar 

  • Remacha L, Currás-Freixes M, Torres-Ruiz R, Schiavi F, Torres-Pérez R, Calsina B, Letón R, Comino-Méndez I, Roldán-Romero JM, Montero-Conde C, Santos M, Pérez LI, Pita G, Alonso MR, Honrado E, Pedrinaci S, Crespo-Facorro B, Percesepe A, Falcioni M, Rodríguez-Perales S, Korpershoek E, Ramón-Maiques S, Opocher G, Rodríguez-Antona C, Robledo M, Cascón A. Gain-of-function mutations in DNMT3A in patients with paraganglioma. Genet Med. 2018 20(12):1644–1651.

    Google Scholar 

  • Roszko KL, Blouch E, Blake M, Powers JF, Tischler AS, Hodin R, Sadow P, Lawson EA. Case report of a prolactinoma in a patient with a novel MAX mutation and bilateral pheochromocytomas. J Endocr Soc. 2017;1(11):1401–7.

    Article  Google Scholar 

  • Schiavi F, Boedeker CC, Bausch B, Peçzkowska M, Gomez CF, Strassburg T, Pawlu C, Buchta M, Salzmann M, Hoffmann MM, Berlis A, Brink I, Cybulla M, Muresan M, Walter MA, Forrer F, Välimäki M, Kawecki A, Szutkowski Z, Schipper J, Walz MK, Pigny P, Bauters C, Willet-Brozick JE, Baysal BE, Januszewicz A, Eng C, Opocher G, Neumann HP, European-American Paraganglioma Study Group. Predictors and prevalence of paraganglioma syndrome associated with mutations of the SDHC gene. JAMA. 2005;294(16):2057–63.

    Article  CAS  Google Scholar 

  • Schiavi F, Milne RL, Anda E, Blay P, Castellano M, Opocher G, Robledo M, Cascón A. Are we overestimating the penetrance of mutations in SDHB? Hum Mutat. 2010;31(6):761–2.

    Article  Google Scholar 

  • Schimke RN, Collins DL, Stolle CA. Paraganglioma, neuroblastoma, and a SDHB mutation: resolution of a 30-year-old mystery. Am J Med Genet A. 2010;152A(6):1531–5.

    PubMed  Google Scholar 

  • Schlisio S, Kenchappa RS, Vredeveld LC, George RE, Stewart R, Greulich H, Shahriari K, Nguyen NV, Pigny P, Dahia PL, Pomeroy SL, Maris JM, Look AT, Meyerson M, Peeper DS, Carter BD, Kaelin WG Jr. The kinesin KIF1Bbeta acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressor. Genes Dev. 2008;22:884–93.

    Article  CAS  Google Scholar 

  • Verine J, Pluvinage A, Bousquet G, Lehmann-Che J, de Bazelaire C, Soufir N, Mongiat-Artus P. Hereditary renal cancer syndromes: an update of a systematic review. Eur Urol. 2010 58(5):701–10.

    Google Scholar 

  • Walther MM, Herring J, Enquist E, Keiser HR, Linehan WM. von Recklinghausen’s disease and pheochromocytomas. J Urol. 1999;162(5):1582–6.

    Article  CAS  Google Scholar 

  • Wells SA Jr, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF, Lee N, Machens A, Moley JF, Pacini F, Raue F, Frank-Raue K, Robinson B, Rosenthal MS, Santoro M, Schlumberger M, Shah M, Waguespack SG, American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid. 2015;25(6):567–610.

    Article  Google Scholar 

  • Yang C, Zhuang Z, Fliedner SM, Shankavaram U, Sun MG, Bullova P, Zhu R, Elkahloun AG, Kourlas PJ, Merino M, Kebebew E, Pacak K. Germ-line PHD1 and PHD2 mutations detected in patients with pheochromocytoma/paraganglioma-polycythemia. J Mol Med (Berl). 2015;93(1):93–104.

    Article  CAS  Google Scholar 

  • Yao L, Schiavi F, Cascon A, Qin Y, Inglada-Pérez L, King EE, Toledo RA, Ercolino T, Rapizzi E, Ricketts CJ, Mori L, Giacchè M, Mendola A, Taschin E, Boaretto F, Loli P, Iacobone M, Rossi GP, Biondi B, Lima-Junior JV, Kater CE, Bex M, Vikkula M, Grossman AB, Gruber SB, Barontini M, Persu A, Castellano M, Toledo SP, Maher ER, Mannelli M, Opocher G, Robledo M, Dahia PL. Spectrum and prevalence of FP/TMEM127 gene mutations in pheochromocytomas and paragangliomas. JAMA. 2010;304:2611–9.

    Article  CAS  Google Scholar 

  • Yeh IT, Lenci RE, Qin Y, Buddavarapu K, Ligon AH, Leteurtre E, Do Cao C, Cardot-Bauters C, Pigny P, Dahia PL. A germline mutation of the KIF1B beta gene on 1p36 in a family with neural and nonneural tumors. Hum Genet. 2008;124:279–85.

    Article  CAS  Google Scholar 

  • Zhuang Z, Yang C, Lorenzo F, Merino M, Fojo T, Kebebew E, Popovic V, Stratakis CA, Prchal JT, Pacak K. Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia. N Engl J Med. 2012;367(10):922–30.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Veneto Institute of Oncology, IRCCS, Padova, Italy

    Giuseppe Opocher

  2. Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Padova, Italy

    Alfonso Massimiliano Ferrara, Stefania Zovato, Giovanni Barbon, Elisa Taschin & Francesca Schiavi

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  1. Giuseppe Opocher
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  2. Alfonso Massimiliano Ferrara
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  3. Stefania Zovato
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  4. Giovanni Barbon
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  5. Elisa Taschin
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  6. Francesca Schiavi
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Correspondence to Giuseppe Opocher .

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  1. Neuroendocrine Unit, University of Naples Federico II Neuroendocrine Unit, Naples, Italy

    Annamaria Colao

  2. Dept. Experimental Medicine, University of L'Aquila Dept. Experimental Medicine, L'Aquila, Italy

    Marie-Lise Jaffrain-Rea

  3. Department of Endocrinology, Centre Hospitalier Universitaire de Lièg Department of Endocrinology, Liège, Belgium

    Albert Beckers

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Opocher, G., Ferrara, A.M., Zovato, S., Barbon, G., Taschin, E., Schiavi, F. (2019). Pheochromocytomas in Complex Genetic Disorders. In: Colao, A., Jaffrain-Rea, ML., Beckers, A. (eds) Polyendocrine Disorders and Endocrine Neoplastic Syndromes. Endocrinology. Springer, Cham. https://doi.org/10.1007/978-3-319-73082-0_14-1

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