{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T22:16:30Z","timestamp":1791756990104,"version":"4.3.4"},"reference-count":109,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2017,6,1]],"date-time":"2017-06-01T00:00:00Z","timestamp":1496275200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,6,1]],"date-time":"2017-06-01T00:00:00Z","timestamp":1496275200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["aacejournalendocrinepractice.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Endocrine Practice"],"published-print":{"date-parts":[[2017,6]]},"DOI":"10.4158\/ep161718.ra","type":"journal-article","created":{"date-parts":[[2017,3,23]],"date-time":"2017-03-23T11:32:12Z","timestamp":1490268732000},"page":"690-704","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":67,"title":["Precision Medicine: An Update on Genotype\/Biochemical Phenotype Relationships in Pheochromocytoma\/Paraganglioma Patients"],"prefix":"10.1016","volume":"23","author":[{"given":"Garima","family":"Gupta","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"on behalf of the AACE Adrenal Scientific Committee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.4158\/EP161718.RA_bb0010","doi-asserted-by":"crossref","first-page":"315","DOI":"10.7326\/0003-4819-134-4-200102200-00016","article-title":"Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma","volume":"134","author":"Pacak","year":"2001","journal-title":"Ann Intern Med"},{"key":"10.4158\/EP161718.RA_bb0015","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/S0140-6736(05)67139-5","article-title":"Phaeochromocytoma","volume":"366","author":"Lenders","year":"2005","journal-title":"Lancet"},{"key":"10.4158\/EP161718.RA_bb0020","doi-asserted-by":"crossref","first-page":"1486","DOI":"10.1373\/clinchem.2014.224832","article-title":"Laboratory evaluation of pheochromocytoma and paraganglioma","volume":"60","author":"Eisenhofer","year":"2014","journal-title":"Clin Chem"},{"key":"10.4158\/EP161718.RA_bb0025","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1373\/clinchem.2004.045484","article-title":"Pheochromocytoma catecholamine phenotypes and prediction of tumor size and location by use of plasma free metanephrines","volume":"51","author":"Eisenhofer","year":"2005","journal-title":"Clin Chem"},{"key":"10.4158\/EP161718.RA_bb0030","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/BF01607135","article-title":"Catecholamine synthesizing enzymes in 70 cases of functioning and nonfunctioning phaeochromocytoma and extra-adrenal paraganglioma","volume":"421","author":"Kimura","year":"1992","journal-title":"Virchows Arch A Pathol Anat Histopathol"},{"key":"10.4158\/EP161718.RA_bb0035","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.1210\/jcem.86.5.7496","article-title":"Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes","volume":"86","author":"Eisenhofer","year":"2001","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0040","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1677\/erc.1.00838","article-title":"Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia-driven angiogenic pathways in von Hippel-Lindau syndrome","volume":"11","author":"Eisenhofer","year":"2004","journal-title":"Endocr Relat Cancer"},{"key":"10.4158\/EP161718.RA_bb0045","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1210\/jc.2004-2025","article-title":"Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine","volume":"90","author":"Eisenhofer","year":"2005","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0050","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1007\/s00268-005-7860-7","article-title":"Dopamine-secreting pheochromocytomas: in search of a syndrome","volume":"29","author":"Dubois","year":"2005","journal-title":"World J Surg"},{"key":"10.4158\/EP161718.RA_bb0055","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1530\/EJE-13-0529","article-title":"Plasma levels of free metanephrines and 3-methoxytyramine indicate a higher number of biochemically active HNPGL than 24-h urinary excretion rates of catecholamines and metabolites","volume":"169","author":"van Duinen","year":"2013","journal-title":"Eur J Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0060","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.1016\/j.ejca.2011.07.016","article-title":"Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status","volume":"48","author":"Eisenhofer","year":"2012","journal-title":"Eur J Cancer"},{"key":"10.4158\/EP161718.RA_bb0065","first-page":"5153","article-title":"Dopamine excess in patients with head and neck paragangliomas","volume":"30","author":"Van Der Horst-Schrivers","year":"2010","journal-title":"Anticancer Res"},{"key":"10.4158\/EP161718.RA_bb0070","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1038\/ncpendmet0396","article-title":"Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005","volume":"3","author":"Pacak","year":"2007","journal-title":"Nat Clin Pract Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0075","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1001\/jama.287.11.1427","article-title":"Biochemical diagnosis of pheochromocytoma: which test is best?","volume":"287","author":"Lenders","year":"2002","journal-title":"JAMA"},{"key":"10.4158\/EP161718.RA_bb0080","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1111\/j.1749-6632.2002.tb04410.x","article-title":"New advances in the biochemical diagnosis of pheochromocytoma: moving beyond catecholamines","volume":"970","author":"Lenders","year":"2002","journal-title":"Ann N Y Acad Sci"},{"key":"10.4158\/EP161718.RA_bb0085","doi-asserted-by":"crossref","first-page":"1100","DOI":"10.1210\/jc.2012-3669","article-title":"Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines?","volume":"98","author":"Stolk","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0090","doi-asserted-by":"crossref","first-page":"8812","DOI":"10.1200\/JCO.2005.03.1484","article-title":"Genetic testing in pheochromocytoma or functional paraganglioma","volume":"23","author":"Amar","year":"2005","journal-title":"J Clin Oncol"},{"key":"10.4158\/EP161718.RA_bb0095","doi-asserted-by":"crossref","first-page":"2729","DOI":"10.1056\/NEJMc066006","article-title":"Clinical and genetic characteristics of patients with neurofibromatosis type 1 and pheochromocytoma","volume":"354","author":"European-American Pheochromocytoma Study G","year":"2006","journal-title":"N Engl J Med"},{"key":"10.4158\/EP161718.RA_bb0100","doi-asserted-by":"crossref","first-page":"5406","DOI":"10.1093\/hmg\/dds402","article-title":"Integrative genomics reveals frequent somatic NF1 mutations in sporadic pheochromocytomas","volume":"21","author":"Welander","year":"2012","journal-title":"Hum Mol Genet"},{"key":"10.4158\/EP161718.RA_bb0105","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1245\/s10434-013-2942-5","article-title":"Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing","volume":"20","author":"Fishbein","year":"2013","journal-title":"Ann Surg Oncol"},{"key":"10.4158\/EP161718.RA_bb0110","doi-asserted-by":"crossref","first-page":"1459","DOI":"10.1056\/NEJMoa020152","article-title":"Germline mutations in nonsyndromic pheochromocytoma","volume":"346","author":"Neumann","year":"2002","journal-title":"N Engl J Med"},{"key":"10.4158\/EP161718.RA_bb0115","doi-asserted-by":"crossref","first-page":"1915","DOI":"10.1210\/jc.2014-1498","article-title":"Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline","volume":"99","author":"Lenders","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0120","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1038\/nrendo.2016.185","article-title":"Consensus Statement on next-generation-sequencing-based diagnostic testing of hereditary phaeochromocytomas and paragangliomas","volume":"13","author":"NGS in PPGL Study Group","year":"2017","journal-title":"Nat Rev Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0125","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1530\/EJE-09-0384","article-title":"Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma","volume":"161","author":"Kopetschke","year":"2009","journal-title":"Eur J Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0130","series-title":"Pheochromocytoma: Diagnosis, Localization, and Treatment","author":"Pacak","year":"2007"},{"key":"10.4158\/EP161718.RA_bb0135","doi-asserted-by":"crossref","first-page":"E1223","DOI":"10.1152\/ajpendo.90591.2008","article-title":"Differential expression of the regulated catecholamine secretory pathway in different hereditary forms of pheochromocytoma","volume":"295","author":"Eisenhofer","year":"2008","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0140","doi-asserted-by":"crossref","first-page":"2175","DOI":"10.1210\/jcem.83.6.4870","article-title":"Plasma metanephrines are markers of pheochromocytoma produced by catechol-O-methyltransferase within tumors","volume":"83","author":"Eisenhofer","year":"1998","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0145","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1196\/annals.1353.026","article-title":"Transcriptional regulation of phenylethanolamine N-methyltransferase in pheochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2","volume":"1073","author":"Huynh","year":"2006","journal-title":"Ann N Y Acad Sci"},{"key":"10.4158\/EP161718.RA_bb0150","first-page":"344","article-title":"Chromogranin A immunoradiometric assay in diagnosis of pheochromocytoma: comparison with plasma metanephrines and 123I-MIBG scan","volume":"50","author":"Giovanella","year":"2006","journal-title":"Q J Nucl Med Mol Imaging"},{"key":"10.4158\/EP161718.RA_bb0155","first-page":"A605","article-title":"What is the source and disposition of chromogranin a (Cga) in normal humanplasma","volume":"35","author":"Oconnor","year":"1987","journal-title":"Clin Res"},{"key":"10.4158\/EP161718.RA_bb0160","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1530\/EJE-06-0640","article-title":"An analysis of the biochemical diagnosis of 66 pheochromocytomas","volume":"156","author":"d'Herbomez","year":"2007","journal-title":"Eur J Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0165","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1507\/endocrj.44.319","article-title":"Plasma chromogranin A in pheochromocytoma, primary hyperparathyroidism and pituitary adenoma in comparison with catecholamine, parathyroid hormone and pituitary hormones","volume":"44","author":"Kimura","year":"1997","journal-title":"Endocr J"},{"key":"10.4158\/EP161718.RA_bb0170","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1046\/j.1365-2265.1999.00628.x","article-title":"Laparoscopic adrenalectomy for phaeochromocytoma: endocrinological and surgical aspects of a new therapeutic approach","volume":"50","author":"Col","year":"1999","journal-title":"Clin Endocrinol (Oxf)"},{"key":"10.4158\/EP161718.RA_bb0175","doi-asserted-by":"crossref","first-page":"4069","DOI":"10.1210\/jc.2007-1720","article-title":"Preoperative management of the pheochromocytoma patient","volume":"92","author":"Pacak","year":"2007","journal-title":"J Clin Endocrinol Metab"},{"issue":"suppl 3","key":"10.4158\/EP161718.RA_bb0180","doi-asserted-by":"crossref","first-page":"S646","DOI":"10.1245\/s10434-015-4862-z","article-title":"Preoperative metyrosine improves cardiovascular outcomes for patients undergoing surgery for pheochromocytoma and paraganglioma","volume":"22","author":"Wachtel","year":"2015","journal-title":"Ann Surg Oncol"},{"key":"10.4158\/EP161718.RA_bb0185","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1097\/00000658-199011000-00010","article-title":"Surgical management of pheochromocytoma with the use of metyrosine","volume":"212","author":"Perry","year":"1990","journal-title":"Ann Surg"},{"key":"10.4158\/EP161718.RA_bb0190","first-page":"449","article-title":"Alternative catecholamine pathways after tyrosine hydroxylase inhibition in malignant pheochromocytoma","volume":"115","author":"Kuchel","year":"1990","journal-title":"J Lab Clin Med"},{"key":"10.4158\/EP161718.RA_bb0195","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1373\/clinchem.2010.153320","article-title":"Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma","volume":"57","author":"Eisenhofer","year":"2011","journal-title":"Clin Chem"},{"key":"10.4158\/EP161718.RA_bb0200","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1007\/BF02067379","article-title":"The role of epinephrine, norepinephrine, and dopamine in blood pressure disturbances in patients with pheochromocytoma","volume":"16","author":"Ito","year":"1992","journal-title":"World J Surg"},{"key":"10.4158\/EP161718.RA_bb0205","doi-asserted-by":"crossref","first-page":"65","DOI":"10.4149\/endo_2011_02_65","article-title":"Phaeochromocytoma: a catecholamine and oxidative stress disorder","volume":"45","author":"Pacak","year":"2011","journal-title":"Endocr Regul"},{"key":"10.4158\/EP161718.RA_bb0210","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.ecl.2011.02.002","article-title":"Hypertension in pheochromocytoma: characteristics and treatment","volume":"40","author":"Zuber","year":"2011","journal-title":"Endocrinol Metab Clin North Am"},{"key":"10.4158\/EP161718.RA_bb0215","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1016\/0895-7061(96)00057-X","article-title":"Mechanisms of orthostatic hypotension and tachycardia in patients with pheochromocytoma","volume":"9","author":"Streeten","year":"1996","journal-title":"Am J Hypertens"},{"key":"10.4158\/EP161718.RA_bb0220","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/0167-5273(95)02302-D","article-title":"Adrenal tumour, congestive heart failure and hemiparesis in an 18-year-old male. A clinical-pathological conference","volume":"49","author":"Goswami","year":"1995","journal-title":"Int J Cardiol"},{"key":"10.4158\/EP161718.RA_bb0225","first-page":"244","article-title":"Pheochromocytoma underlying hypertension, stroke, and dilated cardiomyopathy","volume":"34","author":"Lin","year":"2007","journal-title":"Tex Heart Inst J"},{"key":"10.4158\/EP161718.RA_bb0230","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1007\/s12020-008-9119-1","article-title":"Improvement in renal dysfunction and symptoms after laparoscopic adrenalectomy in a patient with pheochromocytoma complicated by renal dysfunction","volume":"35","author":"Fujiwara","year":"2009","journal-title":"Endocrine"},{"key":"10.4158\/EP161718.RA_bb0235","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1055\/s-2008-1027355","article-title":"Severe bilateral hypertensive retinopathy and optic neuropathy in a patient with pheochromocytoma","volume":"225","author":"Petkou","year":"2008","journal-title":"Klin Monbl Augenheilkd"},{"key":"10.4158\/EP161718.RA_bb0240","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1023\/A:1022981801344","article-title":"Pheochromocytoma as an endocrine emergency","volume":"4","author":"Brouwers","year":"2003","journal-title":"Rev Endocr Metab Disord"},{"key":"10.4158\/EP161718.RA_bb0245","doi-asserted-by":"crossref","first-page":"385","DOI":"10.4158\/EP.11.6.385","article-title":"Bowel perforation as a presenting feature of pheochromocytoma: case report and literature review","volume":"11","author":"Karri","year":"2005","journal-title":"Endocr Pract"},{"key":"10.4158\/EP161718.RA_bb0250","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1530\/EJE-15-0456","article-title":"Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma","volume":"173","author":"Thosani","year":"2015","journal-title":"Eur J Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0255","doi-asserted-by":"crossref","first-page":"1872","DOI":"10.1056\/NEJM199906173402404","article-title":"Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2","volume":"340","author":"Eisenhofer","year":"1999","journal-title":"N Engl J Med"},{"key":"10.4158\/EP161718.RA_bb0260","doi-asserted-by":"crossref","first-page":"1690","DOI":"10.1200\/JCO.2012.47.1912","article-title":"New syndrome of paraganglioma and somatostatinoma associated with polycythemia","volume":"31","author":"Pacak","year":"2013","journal-title":"J Clin Oncol"},{"key":"10.4158\/EP161718.RA_bb0265","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1056\/NEJMoa1205119","article-title":"Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia","volume":"367","author":"Zhuang","year":"2012","journal-title":"N Engl J Med"},{"key":"10.4158\/EP161718.RA_bb0270","doi-asserted-by":"crossref","first-page":"3011","DOI":"10.1093\/hmg\/ddq206","article-title":"SDHA is a tumor suppressor gene causing paraganglioma","volume":"19","author":"Burnichon","year":"2010","journal-title":"Hum Mol Genet"},{"key":"10.4158\/EP161718.RA_bb0275","doi-asserted-by":"crossref","first-page":"E1472","DOI":"10.1210\/jc.2011-1043","article-title":"SDHA immunohistochemistry detects germline SDHA gene mutations in apparently sporadic paragangliomas and pheochromocytomas","volume":"96","author":"Korpershoek","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0280","doi-asserted-by":"crossref","first-page":"E489","DOI":"10.1210\/jc.2013-3486","article-title":"Phenotypic variability and risk of malignancy in SDHC-linked paragangliomas: lessons from three unrelated cases with an identical germline mutation (p.Arg133*)","volume":"99","author":"Bickmann","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0285","doi-asserted-by":"crossref","first-page":"4710","DOI":"10.1210\/jc.2016-1665","article-title":"A SDHC founder mutation causes paragangliomas (PGLs) in the French Canadians: new insights on the SDHC-related PGL","volume":"101","author":"Bourdeau","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0290","doi-asserted-by":"crossref","first-page":"E1482","DOI":"10.1210\/jc.2013-3853","article-title":"The clinical phenotype of SDHC-associated hereditary paraganglioma syndrome (PGL3)","volume":"99","author":"Else","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0295","doi-asserted-by":"crossref","first-page":"190","DOI":"10.4158\/EP15889.OR","article-title":"An aggressive temporal bone SDHC paraganglioma associated with increased HIF-2alpha signaling","volume":"22","author":"Isaacson","year":"2016","journal-title":"Endocr Pract"},{"key":"10.4158\/EP161718.RA_bb0300","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1007\/s12022-013-9296-1","article-title":"Functional cardiac paraganglioma associated with a rare SDHC mutation","volume":"25","author":"Millar","year":"2014","journal-title":"Endocr Pathol"},{"key":"10.4158\/EP161718.RA_bb0305","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1038\/ncponc0935","article-title":"Germline mutations in PTEN and SDHC in a woman with epithelial thyroid cancer and carotid paraganglioma","volume":"4","author":"Zbuk","year":"2007","journal-title":"Nat Clin Pract Oncol"},{"key":"10.4158\/EP161718.RA_bb0310","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1210\/jc.2006-2315","article-title":"Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas","volume":"92","author":"Timmers","year":"2007","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0315","doi-asserted-by":"crossref","first-page":"2440","DOI":"10.1093\/hmg\/ddt639","article-title":"Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas","volume":"23","author":"Castro-Vega","year":"2014","journal-title":"Hum Mol Genet"},{"key":"10.4158\/EP161718.RA_bb0320","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1126\/science.1175689","article-title":"SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma","volume":"325","author":"Hao","year":"2009","journal-title":"Science"},{"key":"10.4158\/EP161718.RA_bb0325","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/S1470-2045(10)70007-3","article-title":"SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma","volume":"11","author":"Bayley","year":"2010","journal-title":"Lancet Oncol"},{"key":"10.4158\/EP161718.RA_bb0330","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1158\/1078-0432.CCR-10-0420","article-title":"SDHAF2 (PGL2-SDH5) and hereditary head and neck paraganglioma","volume":"17","author":"Kunst","year":"2011","journal-title":"Clin Cancer Res"},{"key":"10.4158\/EP161718.RA_bb0335","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1101\/sqb.2011.76.010975","article-title":"Cancer and altered metabolism: potential importance of hypoxia-inducible factor and 2-oxoglutarate-dependent dioxygenases","volume":"76","author":"Kaelin","year":"2011","journal-title":"Cold Spring Harb Symp Quant Biol"},{"key":"10.4158\/EP161718.RA_bb0340","doi-asserted-by":"crossref","first-page":"6044","DOI":"10.1038\/ncomms7044","article-title":"Multi-omics analysis defines core genomic alterations in pheochromocytomas and paragangliomas","volume":"6","author":"Castro-Vega","year":"2015","journal-title":"Nat Commun"},{"key":"10.4158\/EP161718.RA_bb0345","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1111\/j.1471-4159.2009.06153.x","article-title":"Hypoxia inducible factor (HIF)-2 alpha is required for the development of the catecholaminergic phenotype of sympathoadrenal cells","volume":"110","author":"Brown","year":"2009","journal-title":"J Neurochem"},{"key":"10.4158\/EP161718.RA_bb0350","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1096\/fj.02-0445fje","article-title":"Widespread hypoxia-inducible expression of HIF-2alpha in distinct cell populations of different organs","volume":"17","author":"Wiesener","year":"2003","journal-title":"FASEB J"},{"key":"10.4158\/EP161718.RA_bb0355","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1677\/ERC-10-0211","article-title":"Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma","volume":"18","author":"Eisenhofer","year":"2011","journal-title":"Endocr Relat Cancer"},{"key":"10.4158\/EP161718.RA_bb0360","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.ccr.2013.04.018","article-title":"SDH mutations establish a hypermethylator phenotype in paraganglioma","volume":"23","author":"Letouz\u00e9","year":"2013","journal-title":"Cancer Cell"},{"key":"10.4158\/EP161718.RA_bb0365","doi-asserted-by":"crossref","first-page":"e0125426","DOI":"10.1371\/journal.pone.0125426","article-title":"Catecholamine metabolism in paraganglioma and pheochromocytoma: similar tumors in different sites?","volume":"10","author":"Grouzmann","year":"2015","journal-title":"PLoS One"},{"key":"10.4158\/EP161718.RA_bb0370","first-page":"107","article-title":"Whole-exome sequencing identifies MDH2 as a new familial paraganglioma gene","author":"Casc\u00f3n","year":"2015","journal-title":"J Natl Cancer Inst"},{"key":"10.4158\/EP161718.RA_bb0375","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1038\/sj.ejhg.5200793","article-title":"Succinate dehydrogenase and human diseases: new insights into a well-known enzyme","volume":"10","author":"Rustin","year":"2002","journal-title":"Eur J Hum Genet"},{"key":"10.4158\/EP161718.RA_bb0380","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1677\/ERC-09-0024","article-title":"Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas","volume":"16","author":"Havekes","year":"2009","journal-title":"Endocr Relat Cancer"},{"key":"10.4158\/EP161718.RA_bb0385","doi-asserted-by":"crossref","first-page":"E369","DOI":"10.1210\/jc.2013-2600","article-title":"Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome","volume":"99","author":"Buffet","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0390","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1007\/s00109-012-0967-z","article-title":"A novel EPAS1\/HIF2A germline mutation in a congenital polycythemia with paraganglioma","volume":"91","author":"Lorenzo","year":"2013","journal-title":"J Mol Med (Berl)"},{"key":"10.4158\/EP161718.RA_bb0395","doi-asserted-by":"crossref","first-page":"E2046","DOI":"10.1210\/jc.2014-1659","article-title":"Germline FH mutations presenting with pheochromocytoma","volume":"99","author":"Clark","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0400","doi-asserted-by":"crossref","first-page":"E1392","DOI":"10.1210\/jc.2013-4536","article-title":"Universal genetic screening uncovers a novel presentation of an SDHAF2 mutation","volume":"99","author":"Casey","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0405","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1111\/j.1365-2265.1992.tb02326.x","article-title":"Phaeochromocytomas as a cause of hypotension","volume":"37","author":"Baxter","year":"1992","journal-title":"Clin Endocrinol (Oxf)"},{"key":"10.4158\/EP161718.RA_bb0410","doi-asserted-by":"crossref","first-page":"849","DOI":"10.7326\/0003-4819-140-10-200405180-00033","article-title":"Epinephrine-secreting pheochromocytoma presenting with cardiogenic shock and profound hypocalcemia","volume":"140","author":"Olson","year":"2004","journal-title":"Ann Intern Med"},{"key":"10.4158\/EP161718.RA_bb0415","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1530\/eje.0.1470085","article-title":"The value of plasma markers for the clinical behaviour of phaeochromocytomas","volume":"147","author":"van der Harst","year":"2002","journal-title":"Eur J Endocrinol"},{"key":"10.4158\/EP161718.RA_bb0420","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1210\/jcem-54-3-619","article-title":"Steroid enzyme activities in extraadrenal pheochromocytomas","volume":"54","author":"Carballeira","year":"1982","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0425","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1016\/S0022-5347(05)68051-2","article-title":"Enhanced expression of mRNA coding for the adrenaline-synthesizing enzyme phenylethanolamine-N-methyl transferase in adrenaline-secreting pheochromocytomas","volume":"163","author":"Isobe","year":"2000","journal-title":"J Urol"},{"key":"10.4158\/EP161718.RA_bb0430","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s12022-012-9199-6","article-title":"Signaling pathways in pheochromocytomas and paragangliomas: prospects for future therapies","volume":"23","author":"N\u00f6lting","year":"2012","journal-title":"Endocr Pathol"},{"key":"10.4158\/EP161718.RA_bb0435","doi-asserted-by":"crossref","first-page":"3478","DOI":"10.1210\/jc.2006-0780","article-title":"Comprehensive mutation scanning of NF1 in apparently sporadic cases of pheochromocytoma","volume":"91","author":"Bausch","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0440","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1210\/jc.2010-1588","article-title":"Age at diagnosis of pheochromocytoma differs according to catecholamine phenotype and tumor location","volume":"96","author":"Eisenhofer","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0445","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1038\/ng.533","article-title":"Germline mutations in TMEM127 confer susceptibility to pheochromocytoma","volume":"42","author":"Qin","year":"2010","journal-title":"Nat Genet"},{"key":"10.4158\/EP161718.RA_bb0450","doi-asserted-by":"crossref","first-page":"3974","DOI":"10.1093\/hmg\/ddr324","article-title":"Integrative genomic analysis reveals somatic mutations in pheochromocytoma and paraganglioma","volume":"20","author":"Burnichon","year":"2011","journal-title":"Hum Mol Genet"},{"key":"10.4158\/EP161718.RA_bb0455","doi-asserted-by":"crossref","first-page":"E805","DOI":"10.1210\/jc.2011-3360","article-title":"TMEM127 screening in a large cohort of patients with pheochromocytoma and\/or paraganglioma","volume":"97","author":"Abermil","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0460","doi-asserted-by":"crossref","first-page":"E1279","DOI":"10.1210\/jc.2011-0114","article-title":"Germline mutations of the TMEM127 gene in patients with paraganglioma of head and neck and extraadrenal abdominal sites","volume":"96","author":"Neumann","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0465","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1038\/ng.861","article-title":"Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma","volume":"43","author":"Comino-M\u00e9ndez","year":"2011","journal-title":"Nat Genet"},{"key":"10.4158\/EP161718.RA_bb0470","doi-asserted-by":"crossref","first-page":"2828","DOI":"10.1158\/1078-0432.CCR-12-0160","article-title":"MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma","volume":"18","author":"Burnichon","year":"2012","journal-title":"Clin Cancer Res"},{"key":"10.4158\/EP161718.RA_bb0475","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1210\/jc.2015-2592","article-title":"Complex MAX rearrangement in a family with malignant pheochromocytoma, renal oncocytoma, and erythrocytosis","volume":"101","author":"Korpershoek","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0480","doi-asserted-by":"crossref","first-page":"2054","DOI":"10.1002\/ijc.28868","article-title":"Opposing effects of HIF1alpha and HIF2alpha on chromaffin cell phenotypic features and tumor cell proliferation: insights from MYC-associated factor X","volume":"135","author":"Qin","year":"2014","journal-title":"Int J Cancer"},{"key":"10.4158\/EP161718.RA_bb0485","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1006\/jsre.1993.1094","article-title":"Identification of dopamine1A receptors in the rat small intestine","volume":"54","author":"Marmon","year":"1993","journal-title":"J Surg Res"},{"key":"10.4158\/EP161718.RA_bb0490","first-page":"e89","article-title":"Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management","volume":"51","author":"Foo","year":"2010","journal-title":"Singapore Med J"},{"key":"10.4158\/EP161718.RA_bb0495","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1007\/BF03345807","article-title":"Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases","volume":"15","author":"Schlumberger","year":"1992","journal-title":"J Endocrinol Invest"},{"key":"10.4158\/EP161718.RA_bb0500","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1111\/j.1751-7176.2010.00278.x","article-title":"Surgical cure of hypertension in a patient with MEN 2A syndrome and mixed dopamine, metanephrine pheochromocytoma","volume":"12","author":"Figueroa","year":"2010","journal-title":"J Clin Hypertens (Greenwich)"},{"key":"10.4158\/EP161718.RA_bb0505","first-page":"543","article-title":"Dopamine-secreting pheochromocytoma with neurofibromatosis type 1: a case report [in Japanese]","volume":"58","author":"Nakazawa","year":"2012","journal-title":"Hinyokika Kiyo"},{"issue":"suppl 2","key":"10.4158\/EP161718.RA_bb0510","first-page":"43","article-title":"[The von Hippel-Lindau syndrome with pheochromocytoma]","volume":"130","author":"Palmar","year":"2002","journal-title":"Srp Arh Celok Lek"},{"key":"10.4158\/EP161718.RA_bb0515","first-page":"150059","article-title":"Neurofibromatosis-related phaeochromocytoma: two cases with large tumours and elevated plasma methoxytyramine","volume":"2015","author":"Teasdale","year":"2015","journal-title":"Endocrinol Diabetes Metab Case Rep"},{"key":"10.4158\/EP161718.RA_bb0520","doi-asserted-by":"crossref","first-page":"4826","DOI":"10.1210\/jc.2008-1093","article-title":"Biochemically silent abdominal paragangliomas in patients with mutations in the succinate dehydrogenase subunit B gene","volume":"93","author":"Timmers","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0525","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1530\/ERC-11-0369","article-title":"Head and neck paragangliomas: genetic spectrum and clinical variability in 79 consecutive patients","volume":"19","author":"Piccini","year":"2012","journal-title":"Endocr Relat Cancer"},{"key":"10.4158\/EP161718.RA_bb0530","doi-asserted-by":"crossref","first-page":"1938","DOI":"10.1210\/jc.2009-0354","article-title":"Head and neck paragangliomas in von Hippel-Lindau disease and multiple endocrine neoplasia type 2","volume":"94","author":"Boedeker","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0535","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1212\/WNL.37.1.129","article-title":"Multiple paragangliomas in neurofibromatosis: a new neuroendocrine neoplasia","volume":"37","author":"DeAngelis","year":"1987","journal-title":"Neurology"},{"key":"10.4158\/EP161718.RA_bb0540","doi-asserted-by":"crossref","first-page":"4367","DOI":"10.1210\/jc.2009-1479","article-title":"Parasympathetic paragangliomas are part of the Von Hippel-Lindau syndrome","volume":"94","author":"Gaal","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"10.4158\/EP161718.RA_bb0545","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1017\/S0022215100145761","article-title":"Paraganglioma as a systemic syndrome: pitfalls and strategies","volume":"113","author":"Maier","year":"1999","journal-title":"J Laryngol Otol"},{"key":"10.4158\/EP161718.RA_bb0550","first-page":"86","article-title":"Pheochromocytoma: an uncommon presentation of an asymptomatic and biochemically silent adrenal incidentaloma","volume":"19","author":"Kota","year":"2012","journal-title":"Malays J Med Sci"}],"container-title":["Endocrine Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1530891X20358080?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1530891X20358080?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T22:02:27Z","timestamp":1761516147000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1530891X20358080"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6]]},"references-count":109,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2017,6]]}},"alternative-id":["S1530891X20358080"],"URL":"https:\/\/doi.org\/10.4158\/ep161718.ra","relation":{},"ISSN":["1530-891X"],"issn-type":[{"value":"1530-891X","type":"print"}],"subject":[],"published":{"date-parts":[[2017,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Precision Medicine: An Update on Genotype\/Biochemical Phenotype Relationships in Pheochromocytoma\/Paraganglioma Patients","name":"articletitle","label":"Article Title"},{"value":"Endocrine Practice","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.4158\/EP161718.RA","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2017 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}