{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:23:56Z","timestamp":1790821436489,"version":"4.1.0"},"reference-count":171,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2019,10,8]],"date-time":"2019-10-08T00:00:00Z","timestamp":1570492800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["CRC\/Transregio 205\/1 \u201cThe Adrenal: Central Relay in Health and Disease\u201c (314061271-TRR 205; Project No. B10 (M.U., J.P., C.G.Z.) and Project No. B12 (G.E.))"],"award-info":[{"award-number":["CRC\/Transregio 205\/1 \u201cThe Adrenal: Central Relay in Health and Disease\u201c (314061271-TRR 205; Project No. B10 (M.U., J.P., C.G.Z.) and Project No. B12 (G.E.))"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Pheochromocytomas and paragangliomas (PCC\/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC\/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC\/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC\/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine.<\/jats:p>","DOI":"10.3390\/cancers11101505","type":"journal-article","created":{"date-parts":[[2019,10,9]],"date-time":"2019-10-09T03:14:33Z","timestamp":1570590873000},"page":"1505","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":152,"title":["Current Management of Pheochromocytoma\/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine"],"prefix":"10.3390","volume":"11","author":[{"given":"Svenja","family":"N\u00f6lting","sequence":"first","affiliation":[{"name":"Department of Medicine IV, University Hospital, LMU Munich, Ziemssenstra\u00dfe 1, 80336 M\u00fcnchen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6104-6676","authenticated-orcid":false,"given":"Martin","family":"Ullrich","sequence":"additional","affiliation":[{"name":"Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf, Bautzner Landstrasse 400, 01328 Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1610-1493","authenticated-orcid":false,"given":"Jens","family":"Pietzsch","sequence":"additional","affiliation":[{"name":"Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf, Bautzner Landstrasse 400, 01328 Dresden, Germany"},{"name":"Department of Chemistry and Food Chemistry, School of Science, Technische Universit\u00e4t Dresden, Mommsenstrasse 9, 01062 Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian G.","family":"Ziegler","sequence":"additional","affiliation":[{"name":"Department of Medicine III, University Hospital Carl Gustav Carus Dresden, Fetscherstra\u00dfe 74, 01307 Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graeme","family":"Eisenhofer","sequence":"additional","affiliation":[{"name":"Department of Medicine III, University Hospital Carl Gustav Carus Dresden, Fetscherstra\u00dfe 74, 01307 Dresden, Germany"},{"name":"Institute for Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus at Technische Universit\u00e4t Dresden, 01307 Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Grossman","sequence":"additional","affiliation":[{"name":"Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford Ox3 7LJ, UK"},{"name":"Department of Gastroenterology, Royal Free Hospital ENETS Centre of Excellence, London NW3 2QG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20814, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,10,8]]},"reference":[{"key":"ref_1","first-page":"802","article-title":"Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979","volume":"58","author":"Beard","year":"1983","journal-title":"Mayo Clin. Proc."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.ccell.2017.01.001","article-title":"Comprehensive molecular characterization of pheochromocytoma and paraganglioma","volume":"31","author":"Fishbein","year":"2017","journal-title":"Cancer Cell"},{"key":"ref_3","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":"ref_4","first-page":"354","article-title":"Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series","volume":"56","author":"Sutton","year":"1981","journal-title":"Mayo Clin. Proc."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1210\/jc.2016-3829","article-title":"Characteristics of pediatric vs adult pheochromocytomas and paragangliomas","volume":"102","author":"Pamporaki","year":"2017","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1646","DOI":"10.1373\/clinchem.2018.291369","article-title":"Biochemical diagnosis of chromaffin cell tumors in patients at high and low risk of disease: Plasma versus urinary free or deconjugated O-methylated catecholamine metabolites","volume":"64","author":"Eisenhofer","year":"2018","journal-title":"Clin. Chem."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Geroula, A., Deutschbein, T., Langton, K., Masjkur, J.R., Pamporaki, C., Peitzsch, M., Fliedner, S., Timmers, H.J., Bornstein, S.R., and Beuschlein, F. (2019). Pheochromocytoma and paraganglioma: Clinical feature based disease probability in relation to catecholamine biochemistry and reason for disease suspicion. Eur. J. Endocrinol.","DOI":"10.1530\/EJE-19-0159"},{"key":"ref_8","first-page":"69","article-title":"Metabologenomics of phaeochromocytoma and paraganglioma: An integrated approach for personalised biochemical and genetic testing","volume":"38","author":"Eisenhofer","year":"2017","journal-title":"Clin. Biochem. Rev."},{"key":"ref_9","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":"ref_10","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1097\/00000658-197405000-00029","article-title":"Current management of pheochromocytoma","volume":"179","author":"Remine","year":"1974","journal-title":"Ann. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1007\/BF00353738","article-title":"\u201cThe\u201d pheochromocytoma: A benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist?","volume":"18","author":"Proye","year":"1994","journal-title":"World J. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1097\/00000658-199906000-00001","article-title":"Clinical experience over 48 years with pheochromocytoma","volume":"229","author":"Goldstein","year":"1999","journal-title":"Ann. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1530\/eje.0.1410619","article-title":"Pheochromocytoma in Italy: A multicentric retrospective study","volume":"141","author":"Mannelli","year":"1999","journal-title":"Eur. J. Endocrinol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1016\/S0090-4295(98)00612-8","article-title":"Pheochromocytomas: Can malignant potential be predicted?","volume":"53","author":"John","year":"1999","journal-title":"Urology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1053\/ejso.2002.1413","article-title":"The management of benign and malignant pheochromocytoma and abdominal paraganglioma","volume":"29","author":"Elder","year":"2003","journal-title":"Eur. J. Surg. Oncol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"2110","DOI":"10.1210\/jc.2004-1398","article-title":"Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma","volume":"90","author":"Amar","year":"2005","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_17","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":"ref_18","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1111\/cen.13434","article-title":"Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: A systematic review and meta-analysis","volume":"87","author":"Hamidi","year":"2017","journal-title":"Clin. Endocrinol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1007\/s12022-017-9484-5","article-title":"Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours","volume":"28","author":"Lam","year":"2017","journal-title":"Endocr. Pathol."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Asa, S.L., Ezzat, S., and Mete, O. (2018). The diagnosis and clinical significance of paragangliomas in unusual locations. J. Clin. Med., 7.","DOI":"10.3390\/jcm7090280"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1111\/jgh.12254","article-title":"Education and imaging. Hepatobiliary and pancreatic: A huge liver paraganglioma","volume":"28","author":"Koh","year":"2013","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e0293","DOI":"10.1097\/MD.0000000000010293","article-title":"Primary functioning hepatic paraganglioma mimicking hepatocellular carcinoma: A case report and literature review","volume":"97","author":"Liao","year":"2018","journal-title":"Medicine"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1007\/s12022-013-9258-7","article-title":"Endobronchial gangliocytic paraganglioma: Not all keratin-positive endobronchial neuroendocrine neoplasms are pulmonary carcinoids","volume":"25","author":"Gucer","year":"2014","journal-title":"Endocr. Pathol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e1271","DOI":"10.1097\/MD.0000000000001271","article-title":"Primary pulmonary paraganglioma: A case report and review of literature","volume":"94","author":"Huang","year":"2015","journal-title":"Medicine"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1186\/s13256-015-0639-z","article-title":"Primary paraganglioma of the lung: A case report","volume":"9","author":"Fiorentino","year":"2015","journal-title":"J. Med. Case Rep."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1097\/00000478-200205000-00002","article-title":"Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: A clinicopathologic and immunophenotypic study of 100 cases","volume":"26","author":"Thompson","year":"2002","journal-title":"Am J. Surg. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1530\/ERC-13-0494","article-title":"Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma","volume":"21","author":"Kimura","year":"2014","journal-title":"Endocr. Relat. Cancer"},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Kimura, N., Takekoshi, K., and Naruse, M. (2018). Risk stratification on pheochromocytoma and paraganglioma from laboratory and clinical medicine. J. Clin. Med., 7.","DOI":"10.20944\/preprints201807.0478.v1"},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Stenman, A., Zedenius, J., and Juhlin, C.C. (2019). The value of histological algorithms to predict the malignancy potential of pheochromocytomas and abdominal paragangliomas\u2014A meta-analysis and systematic review of the literature. Cancers, 11.","DOI":"10.3390\/cancers11020225"},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Stenman, A., Zedenius, J., and Juhlin, C.C. (2019). Retrospective application of the pathologic tumor-node-metastasis classification system for pheochromocytoma and abdominal paraganglioma in a well characterized cohort with long-term follow-up. Surgery.","DOI":"10.1016\/j.surg.2019.04.030"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1097\/MED.0000000000000330","article-title":"Malignant pheochromocytoma-paraganglioma: Pathogenesis, TNM staging, and current clinical trials","volume":"24","author":"Jimenez","year":"2017","journal-title":"Curr. Opin. Endocrinol. Diabetes Obes."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1210\/er.2017-00062","article-title":"New perspectives on pheochromocytoma and paraganglioma: Toward a molecular classification","volume":"38","author":"Crona","year":"2017","journal-title":"Endocr. Rev."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/j.trecan.2017.11.001","article-title":"Genomic landscape of pheochromocytoma and paraganglioma","volume":"4","author":"Jochmanova","year":"2018","journal-title":"Trends Cancer"},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"N\u00f6lting, S., Grossman, A., and Pacak, K. (2018). Metastatic Phaeochromocytoma: Spinning towards more promising treatment options. Exp. Clin. Endocrinol. Diabetes.","DOI":"10.1055\/a-0715-1888"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1093\/jnci\/djt201","article-title":"Hypoxia-inducible factor signaling in pheochromocytoma: Turning the rudder in the right direction","volume":"105","author":"Jochmanova","year":"2013","journal-title":"J. Natl. Cancer Inst."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ejca.2017.08.023","article-title":"HIF-2alpha: Achilles\u2019 heel of pseudohypoxic subtype paraganglioma and other related conditions","volume":"86","author":"Tella","year":"2017","journal-title":"Eur. J. Cancer"},{"key":"ref_37","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":"ref_38","doi-asserted-by":"crossref","first-page":"2054","DOI":"10.1002\/ijc.28868","article-title":"Opposing effects of HIF1\u03b1 and HIF2\u03b1 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":"ref_39","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-16-0033","article-title":"European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma","volume":"174","author":"Plouin","year":"2016","journal-title":"Eur. J. Endocrinol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1089\/thy.2015.0020","article-title":"2015 American Thyroid Association management guidelines for adult patients with thyroid nodules and differentiated thyroid cancer: The American Thyroid Association guidelines task force on thyroid nodules and differentiated thyroid cancer","volume":"26","author":"Haugen","year":"2016","journal-title":"Thyroid"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.3201\/eid0706.010618","article-title":"Vancomycin-intermediate Staphylococcus aureus in a home health-care patient","volume":"7","author":"Hageman","year":"2001","journal-title":"Emerg. Infect. Dis."},{"key":"ref_42","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":"ref_43","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1111\/cen.12327","article-title":"Biochemical diagnosis of phaeochromocytoma using plasma-free normetanephrine, metanephrine and methoxytyramine: Importance of supine sampling under fasting conditions","volume":"80","author":"Darr","year":"2014","journal-title":"Clin. Endocrinol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1530\/EJE-14-0730","article-title":"Measurements of plasma metanephrines by immunoassay vs liquid chromatography with tandem mass spectrometry for diagnosis of pheochromocytoma","volume":"172","author":"Weismann","year":"2015","journal-title":"Eur. J. Endocrinol."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Boyd, J., Leung, A.A., Sadrzadeh, H., Pamporaki, C., Pacak, K., Deutschbein, T., Fliedner, S., and Kline, G.A. (2019). A high rate of modestly elevated plasma normetanephrine in a population referred for suspected PPGL when measured in a seated position. Eur. J. Endocrinol.","DOI":"10.1530\/EJE-19-0176"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1530\/EJE-15-0651","article-title":"Longitudinal plasma metanephrines preceding pheochromocytoma diagnosis: A retrospective case-control serum repository study","volume":"174","author":"Olson","year":"2016","journal-title":"Eur. J. Endocrinol."},{"key":"ref_47","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":"ref_48","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1530\/EJE-14-0756","article-title":"Lack of utility of SDHB mutation testing in adrenergic metastatic phaeochromocytoma","volume":"172","author":"Sue","year":"2015","journal-title":"Eur. J. Endocrinol."},{"key":"ref_49","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":"ref_50","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1093\/ajcp\/72.2.175","article-title":"Deficiency of dopamine-\u03b2-hydroxylase: A new mechanism for normotensive pheochromocytomas","volume":"72","author":"Feldman","year":"1979","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1111\/eci.12245","article-title":"Clinical utility of chromogranin A in SDHx-related paragangliomas","volume":"44","author":"Zuber","year":"2014","journal-title":"Eur. J. Clin. Investig."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1097\/00005792-199101000-00003","article-title":"Chromogranin A storage and secretion: Sensitivity and specificity for the diagnosis of pheochromocytoma","volume":"70","author":"Hsiao","year":"1991","journal-title":"Medicine"},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Bilek, R., Vlcek, P., Safarik, L., Michalsky, D., Novak, K., Duskova, J., Vaclavikova, E., Widimsky, J., and Zelinka, T. (2019). Chromogranin A in the laboratory diagnosis of pheochromocytoma and paraganglioma. Cancers, 11.","DOI":"10.3390\/cancers11040586"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1111\/j.1365-2796.2004.01425.x","article-title":"Biochemical diagnosis, localization and management of pheochromocytoma: Focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour","volume":"257","author":"Pacak","year":"2005","journal-title":"J. Intern. Med."},{"key":"ref_55","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":"ref_56","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1210\/jcem.87.5.8446","article-title":"Utility of plasma free metanephrines for detecting childhood pheochromocytoma","volume":"87","author":"Weise","year":"2002","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_57","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":"ref_58","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":"ref_59","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1080\/110241598750004913","article-title":"Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: A review of 282 cases. French Association of Surgery (AFC), and The French Association of Endocrine Surgeons (AFCE)","volume":"164","author":"Jalil","year":"1998","journal-title":"Eur. J. Surg."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/0002-9343(79)90064-0","article-title":"Diagnosis and localization of pheochromocytoma: Detection by measurement of urinary norepinephrine excretion during sleep, plasma norepinephrine concentration and computerized axial tomography (CT-scan)","volume":"67","author":"Ganguly","year":"1979","journal-title":"Am. J. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"3888","DOI":"10.1158\/1078-0432.CCR-14-2751","article-title":"Superiority of [68Ga]-DOTATATE PET\/CT to other functional imaging modalities in the localization of SDHB-associated metastatic pheochromocytoma and paraganglioma","volume":"21","author":"Janssen","year":"2015","journal-title":"Clin. Cancer Res."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1093\/jnci\/djs188","article-title":"Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography","volume":"104","author":"Timmers","year":"2012","journal-title":"J. Natl. Cancer Inst."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"369","DOI":"10.2967\/jnumed.118.211706","article-title":"Performance of 68Ga-DOTA\u2013Conjugated somatostatin receptor\u2013targeting peptide PET in detection of pheochromocytoma and paraganglioma: A systematic review and metaanalysis","volume":"60","author":"Han","year":"2019","journal-title":"J. Nucl. Med."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"2112","DOI":"10.1007\/s00259-019-04398-1","article-title":"European association of nuclear medicine practice guideline\/society of nuclear medicine and molecular imaging procedure standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma","volume":"46","author":"Taieb","year":"2019","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"53","DOI":"10.3389\/fonc.2019.00053","article-title":"Clinical, diagnostic, and treatment characteristics of SDHA-related metastatic pheochromocytoma and paraganglioma","volume":"9","author":"Jha","year":"2019","journal-title":"Front. Oncol."},{"key":"ref_66","first-page":"46","article-title":"Superiority of 68Ga-DOTATATE PET\/CT to other functional and anatomic imaging modalities in the detection of SDHD-related pheochromocytoma and paraganglioma\u2014A comparative prospective study","volume":"59","author":"Jha","year":"2018","journal-title":"J. Nucl. Med."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1007\/s00259-017-3896-9","article-title":"Superiority of 68Ga-DOTATATE over 18F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx)-related pheochromocytoma and paraganglioma in the pediatric population","volume":"45","author":"Jha","year":"2018","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"186","DOI":"10.2967\/jnumed.115.161018","article-title":"68Ga-DOTATATE PET\/CT in the localization of head and neck paragangliomas compared with other functional imaging modalities and CT\/MRI","volume":"57","author":"Janssen","year":"2016","journal-title":"J. Nucl. Med."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1007\/s00259-015-3268-2","article-title":"Prospective comparison of 68Ga-DOTATATE and 18F-FDOPA PET\/CT in patients with various pheochromocytomas and paragangliomas with emphasis on sporadic cases","volume":"43","author":"Archier","year":"2016","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1800","DOI":"10.1007\/s00259-013-2548-y","article-title":"A retrospective comparison between 68Ga-DOTA-TOC PET\/CT and 18F-DOPA PET\/CT in patients with extra-adrenal paraganglioma","volume":"40","author":"Kroiss","year":"2013","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1007\/s00259-016-3357-x","article-title":"PET\/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT\/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma","volume":"43","author":"Janssen","year":"2016","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1530\/ERC-16-0231","article-title":"Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome","volume":"23","author":"Darr","year":"2016","journal-title":"Endocr. Relat. Cancer"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.2967\/jnumed.116.187690","article-title":"Functional imaging signature of patients presenting with polycythemia\/paraganglioma syndromes","volume":"58","author":"Janssen","year":"2017","journal-title":"J. Nucl. Med."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1574","DOI":"10.1210\/jc.2017-02324","article-title":"18F-FDOPA PET\/CT imaging of MAX-related pheochromocytoma","volume":"103","author":"Taieb","year":"2018","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"e176","DOI":"10.4158\/EP15759.CR","article-title":"Functional imaging experience in a germline fumarate hydratase mutation\u2013positive patient with pheochromocytoma and paraganglioma","volume":"2","author":"Nambuba","year":"2015","journal-title":"AACE Clin. Case Rep."},{"key":"ref_76","doi-asserted-by":"crossref","unstructured":"Gild, M.L., Naik, N., Hoang, J., Hsiao, E., McGrath, R.T., Sywak, M., Sidhu, S., Delbridge, L.W., Robinson, B.G., and Schembri, G. (2018). Role of DOTATATE-PET\/CT in preoperative assessment of phaeochromocytoma and paragangliomas. Clin. Endocrinol.","DOI":"10.1111\/cen.13737"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-16-0467","article-title":"Management of adrenal incidentalomas: European society of endocrinology clinical practice guideline in collaboration with the European network for the study of adrenal tumors","volume":"175","author":"Fassnacht","year":"2016","journal-title":"Eur. J. Endocrinol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1016\/j.surg.2009.09.013","article-title":"Biopsy of pheochromocytomas and paragangliomas: Potential for disaster","volume":"146","author":"Vanderveen","year":"2009","journal-title":"Surgery"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1530\/ERC-17-0306","article-title":"TERT structural rearrangements in metastatic pheochromocytomas","volume":"25","author":"Dwight","year":"2018","journal-title":"Endocr. Relat. Cancer"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s12022-017-9479-2","article-title":"Malignancy in pheochromocytoma or paraganglioma: Integrative analysis of 176 cases in TCGA","volume":"28","author":"Suh","year":"2017","journal-title":"Endocr. Pathol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1097\/PAS.0000000000001190","article-title":"Molecular profiling of pheochromocytoma and abdominal paraganglioma stratified by the PASS algorithm reveals chromogranin B as associated with histologic prediction of malignant behavior","volume":"43","author":"Stenman","year":"2019","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1111\/cen.13019","article-title":"ERBB-2 overexpression as a risk factor for malignant phaeochromocytomas and paraganglinomas","volume":"84","author":"Wang","year":"2016","journal-title":"Clin. Endocrinol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.humpath.2017.10.013","article-title":"The utility of SDHB and FH immunohistochemistry in patients evaluated for hereditary paraganglioma-pheochromocytoma syndromes","volume":"71","author":"Udager","year":"2018","journal-title":"Hum. Pathol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1007\/s002689900139","article-title":"Clinical spectrum and outcome of functional extraadrenal paraganglioma","volume":"20","author":"Young","year":"1996","journal-title":"World J. Surg."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"4137","DOI":"10.1200\/JCO.2011.34.6353","article-title":"Metastatic pheochromocytoma\/paraganglioma related to primary tumor development in childhood or adolescence: Significant link to SDHB mutations","volume":"29","author":"King","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1196\/annals.1353.038","article-title":"Biochemical diagnosis and localization of pheochromocytoma: Can we reach a consensus?","volume":"1073","author":"Grossman","year":"2006","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1007\/s12020-011-9545-3","article-title":"Pheochromocytomas and paragangliomas: Assessment of malignant potential","volume":"40","author":"Korevaar","year":"2011","journal-title":"Endocrine"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1530\/ERC-13-0415","article-title":"Long-term prognosis of patients with pediatric pheochromocytoma","volume":"21","author":"Bausch","year":"2014","journal-title":"Endocr. Relat. Cancer"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/nrendo.2014.188","article-title":"Paraganglioma and phaeochromocytoma: From genetics to personalized medicine","volume":"11","author":"Favier","year":"2015","journal-title":"Nat. Rev. Endocrinol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1089\/thy.2014.0335","article-title":"Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma","volume":"25","author":"Wells","year":"2015","journal-title":"Thyroid"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"2172","DOI":"10.1200\/JCO.2015.65.6140","article-title":"Von Hippel-Lindau disease: Genetics and role of genetic counseling in a multiple neoplasia syndrome","volume":"34","author":"Nielsen","year":"2016","journal-title":"J. Clin. Oncol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"e68","DOI":"10.1158\/1078-0432.CCR-17-0547","article-title":"Von Hippel-Lindau and hereditary pheochromocytoma\/paraganglioma syndromes: Clinical features, genetics, and surveillance recommendations in childhood","volume":"23","author":"Rednam","year":"2017","journal-title":"Clin. Cancer Res."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1001\/jama.1997.03550010065042","article-title":"The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2","volume":"278","author":"Gutmann","year":"1997","journal-title":"JAMA"},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"5658","DOI":"10.1210\/jcem.86.12.8070","article-title":"Guidelines for diagnosis and therapy of MEN type 1 and type 2","volume":"86","author":"Brandi","year":"2001","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"1109","DOI":"10.1210\/jc.2018-02411","article-title":"Positive impact of genetic test on the management and outcome of patients with paraganglioma and\/or pheochromocytoma","volume":"104","author":"Buffet","year":"2019","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1016\/j.surg.2006.07.039","article-title":"Posterior retroperitoneoscopic adrenalectomy\u2014Results of 560 procedures in 520 patients","volume":"140","author":"Walz","year":"2006","journal-title":"Surgery"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1016\/S1470-2045(14)70154-8","article-title":"Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: An international retrospective population-based study","volume":"15","author":"Castinetti","year":"2014","journal-title":"Lancet Oncol."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1177\/0194599815627667","article-title":"Head and neck paragangliomas: An update on evaluation and management","volume":"154","author":"Moore","year":"2016","journal-title":"Otolaryngol. Head Neck Surg."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"R291","DOI":"10.1530\/ERC-13-0223","article-title":"The management of head-and-neck paragangliomas","volume":"20","author":"Capatina","year":"2013","journal-title":"Endocr. Relat. Cancer"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/j.wneu.2016.10.132","article-title":"Radiosurgery for paragangliomas of the head and neck: Another step for the validation of a treatment paradigm","volume":"98","author":"Marchetti","year":"2017","journal-title":"World Neurosurg."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/j.ijrobp.2014.02.010","article-title":"Treatment of head and neck paragangliomas with external beam radiation therapy","volume":"89","author":"Dupin","year":"2014","journal-title":"Int. J. Radiat. Oncol. Biol. Phys."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"166","DOI":"10.3389\/fonc.2014.00166","article-title":"External beam radiation therapy in treatment of malignant pheochromocytoma and paraganglioma","volume":"4","author":"Vogel","year":"2014","journal-title":"Front. Oncol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"e198898","DOI":"10.1001\/jamanetworkopen.2019.8898","article-title":"Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy","volume":"2","author":"Neumann","year":"2019","journal-title":"JAMA Netw. Open"},{"key":"ref_104","doi-asserted-by":"crossref","unstructured":"Roman-Gonzalez, A., Zhou, S., Ayala-Ramirez, M., Shen, C., Waguespack, S.G., Habra, M.A., Karam, J.A., Perrier, N., Wood, C.G., and Jimenez, C. (2017). Impact of surgical resection of the primary tumor on overall survival in patients with metastatic pheochromocytoma or sympathetic paraganglioma. Ann. Surg.","DOI":"10.1097\/SLA.0000000000002195"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"2804","DOI":"10.1002\/cncr.26577","article-title":"Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: Insights from the largest single-institutional experience","volume":"118","author":"Feng","year":"2012","journal-title":"Cancer"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"3296","DOI":"10.1210\/jc.2017-00992","article-title":"Malignant pheochromocytoma and paraganglioma: 272 patients over 55 years","volume":"102","author":"Hamidi","year":"2017","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"1546","DOI":"10.1245\/s10434-016-5739-5","article-title":"Surgical treatment of malignant pheochromocytoma and paraganglioma: Retrospective case series","volume":"24","author":"Strajina","year":"2017","journal-title":"Ann. Surg. Oncol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"S183","DOI":"10.4103\/0973-1482.122522","article-title":"Surgical resection of multiple liver metastasis of functional malignant pheochromocytoma: A case report and literature review","volume":"9","author":"Wei","year":"2013","journal-title":"J. Cancer Res. Ther."},{"key":"ref_109","first-page":"e447","article-title":"Complete remission in metastatic pheochromocytoma treated with extensive surgery","volume":"8","author":"Sanchez","year":"2016","journal-title":"Cureus"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.adro.2017.11.002","article-title":"External beam radiation therapy for advanced\/unresectable malignant paraganglioma and pheochromocytoma","volume":"3","author":"Breen","year":"2018","journal-title":"Adv. Radiat. Oncol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/s12020-017-1515-y","article-title":"Prevention of serious skeletal-related events by interventional radiology techniques in patients with malignant paraganglioma and pheochromocytoma","volume":"59","author":"Gravel","year":"2018","journal-title":"Endocrine"},{"key":"ref_112","doi-asserted-by":"crossref","unstructured":"Kohlenberg, J., Welch, B., Hamidi, O., Callstrom, M., Morris, J., Sprung, J., Bancos, I., and Young, W. (2019). Efficacy and safety of ablative therapy in the treatment of patients with metastatic pheochromocytoma and paraganglioma. Cancers, 11.","DOI":"10.3390\/cancers11020195"},{"key":"ref_113","first-page":"1480","article-title":"Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: Analysis of 165 operations at a single center","volume":"86","author":"Plouin","year":"2001","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_114","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."},{"key":"ref_115","first-page":"190","article-title":"Pheochromocytoma: A review on preoperative treatment with phenoxybenzamine or doxazosin","volume":"72","year":"2014","journal-title":"Neth. J. Med."},{"key":"ref_116","doi-asserted-by":"crossref","unstructured":"Mak, I.Y.F., Hayes, A.R., Khoo, B., and Grossman, A. (2019). Peptide receptor radionuclide therapy as a novel treatment for metastatic and invasive phaeochromocytoma and paraganglioma. Neuroendocrinology.","DOI":"10.1159\/000499497"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"623","DOI":"10.2967\/jnumed.118.217463","article-title":"Efficacy and safety of high-specific-activity 131I-MIBG therapy in patients with advanced pheochromocytoma or paraganglioma","volume":"60","author":"Pryma","year":"2019","journal-title":"J. Nucl. Med."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1111\/cen.12341","article-title":"131I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: Systematic review and meta-analysis","volume":"80","author":"Niemeijer","year":"2014","journal-title":"Clin. Endocrinol."},{"key":"ref_119","first-page":"100","article-title":"131I-MIBG treatment of pheochromocytoma: Low versus intermediate activity regimens of therapy","volume":"54","author":"Castellani","year":"2010","journal-title":"Q. J. Nucl. Med. Mol. Imaging"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1038\/bjc.2013.365","article-title":"Sequelae and survivorship in patients treated with 131I-MIBG therapy","volume":"109","author":"Sze","year":"2013","journal-title":"Br. J. Cancer"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"15879","DOI":"10.1073\/pnas.0907843106","article-title":"Expression of neuropeptide hormone receptors in human adrenal tumors and cell lines: Antiproliferative effects of peptide analogues","volume":"106","author":"Ziegler","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1080\/02841860701441848","article-title":"Peptide receptor radionuclide therapy with radiolabelled somatostatin analogues in patients with somatostatin receptor positive tumours","volume":"46","author":"Krenning","year":"2007","journal-title":"Acta Oncol."},{"key":"ref_123","first-page":"1599","article-title":"Effects of therapy with [177Lu-DOTA0, Tyr3] octreotate in patients with paraganglioma, meningioma, small cell lung carcinoma, and melanoma","volume":"47","author":"Krenning","year":"2006","journal-title":"J. Nucl. Med."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1055\/s-0032-1311637","article-title":"Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE in individuals with neck or mediastinal paraganglioma (PGL)","volume":"44","author":"Zovato","year":"2012","journal-title":"Horm. Metab. Res."},{"key":"ref_125","first-page":"334","article-title":"Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma","volume":"52","author":"Forrer","year":"2008","journal-title":"Q. J. Nucl. Med. Mol. Imaging"},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"3278","DOI":"10.1210\/jc.2017-00816","article-title":"Efficacy of peptide receptor radionuclide therapy for functional metastatic paraganglioma and pheochromocytoma","volume":"102","author":"Kong","year":"2017","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1002\/jso.24553","article-title":"Peptide receptor radionuclide treatment and 131I-MIBG in the management of patients with metastatic\/progressive phaeochromocytomas and paragangliomas","volume":"115","author":"Nastos","year":"2017","journal-title":"J. Surg. Oncol."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1007\/s12032-016-0737-9","article-title":"Peptide receptor radionuclide therapy for metastatic paragangliomas","volume":"33","author":"Pinato","year":"2016","journal-title":"Med. Oncol."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"2416","DOI":"10.1200\/JCO.2010.33.7873","article-title":"Response, survival, and long-term toxicity after therapy with the radiolabeled somatostatin analogue [90Y-DOTA]-TOC in metastasized neuroendocrine cancers","volume":"29","author":"Imhof","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1007\/s00259-015-3029-2","article-title":"Peptide receptor radionuclide therapy with 90Y\/177Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours)","volume":"42","author":"Puranik","year":"2015","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/s13550-019-0484-y","article-title":"Concomitant 177Lu-DOTATATE and capecitabine therapy in malignant paragangliomas","volume":"9","author":"Yadav","year":"2019","journal-title":"EJNMMI Res."},{"key":"ref_132","first-page":"155","article-title":"Peptide receptor radionuclide therapy in head and neck paragangliomas\u2014Report of 14 cases","volume":"136","author":"Estevao","year":"2015","journal-title":"Rev. Laryngol. Otol. Rhinol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1056\/NEJMoa1607427","article-title":"Phase 3 trial of 177Lu-DOTATATE for midgut neuroendocrine tumors","volume":"376","author":"Strosberg","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1159\/000494760","article-title":"Targeted alpha particle therapy for neuroendocrine tumours: The next generation of peptide receptor radionuclide therapy","volume":"108","author":"Navalkissoor","year":"2019","journal-title":"Neuroendocrinology"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"4617","DOI":"10.1158\/1078-0432.CCR-16-2743","article-title":"Long-term efficacy, survival, and safety of [177Lu-DOTA0,Tyr3]octreotate in patients with gastroenteropancreatic and bronchial neuroendocrine tumors","volume":"23","author":"Brabander","year":"2017","journal-title":"Clin. Cancer Res."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/j.thorsurg.2014.04.005","article-title":"Peptide receptor radionuclide therapy for advanced neuroendocrine tumors","volume":"24","author":"Bodei","year":"2014","journal-title":"Thorac. Surg. Clin."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.cpet.2013.10.001","article-title":"Yttrium-based therapy for neuroendocrine tumors","volume":"9","author":"Bodei","year":"2014","journal-title":"PET Clin."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"452","DOI":"10.2967\/jnumed.117.189712","article-title":"Persistent hematologic dysfunction after peptide receptor radionuclide therapy with 177Lu-DOTATATE: Incidence, course, and predicting factors in patients with gastroenteropancreatic neuroendocrine tumors","volume":"59","author":"Bergsma","year":"2018","journal-title":"J. Nucl. Med."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1007\/978-3-642-27994-2_30","article-title":"The Bad Berka dose protocol: Comparative results of dosimetry in peptide receptor radionuclide therapy using 177Lu-DOTATATE, 177Lu-DOTANOC, and 177Lu-DOTATOC","volume":"Volume 194","author":"Schuchardt","year":"2013","journal-title":"Theranostics, Gallium-68, and Other Radionuclides"},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1007\/s00259-018-4209-7","article-title":"Personalized 177Lu-octreotate peptide receptor radionuclide therapy of neuroendocrine tumours: Initial results from the P-PRRT trial","volume":"46","author":"Buteau","year":"2019","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.cpet.2015.06.001","article-title":"Application and dosimetric requirements for gallium-68\u2013labeled somatostatin analogues in targeted radionuclide therapy for gastroenteropancreatic neuroendocrine tumors","volume":"10","author":"Taieb","year":"2015","journal-title":"PET Clin."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13550-018-0443-z","article-title":"Dosimetry methods and clinical applications in peptide receptor radionuclide therapy for neuroendocrine tumours: A literature review","volume":"8","author":"Huizing","year":"2018","journal-title":"EJNMMI Res."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"4656","DOI":"10.1200\/JCO.2009.22.8510","article-title":"Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: A report from the PROMID Study Group","volume":"27","author":"Rinke","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1056\/NEJMoa1316158","article-title":"Lanreotide in metastatic enteropancreatic neuroendocrine tumors","volume":"371","author":"Caplin","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1111\/cen.12542","article-title":"Chemotherapy with cyclophosphamide, vincristine and dacarbazine for malignant paraganglioma and pheochromocytoma: Systematic review and meta-analysis","volume":"81","author":"Niemeijer","year":"2014","journal-title":"Clin. Endocrinol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"267","DOI":"10.7326\/0003-4819-109-4-267","article-title":"Malignant pheochromocytoma: Effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine","volume":"109","author":"Averbuch","year":"1988","journal-title":"Ann. Inter. Med."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"2020","DOI":"10.1002\/cncr.23812","article-title":"Treatment of malignant pheochromocytoma\/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: Recommendation from a 22-year follow-up of 18 patients","volume":"113","author":"Huang","year":"2008","journal-title":"Cancer"},{"key":"ref_148","doi-asserted-by":"crossref","unstructured":"Jawed, I., Velarde, M., Darr, R., Wolf, K.I., Adams, K., Venkatesan, A.M., Balasubramaniam, S., Poruchynsky, M.S., Reynolds, J.C., and Pacak, K. (2018). Continued tumor reduction of metastatic pheochromocytoma\/paraganglioma harboring succinate dehydrogenase subunit B mutations with cyclical chemotherapy. Cell. Mol. Neurobiol.","DOI":"10.1007\/s10571-018-0579-4"},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"2711","DOI":"10.1002\/ijc.28913","article-title":"SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma","volume":"135","author":"Hadoux","year":"2014","journal-title":"Int. J. Cancer"},{"key":"ref_150","doi-asserted-by":"crossref","unstructured":"Tena, I., Gupta, G., Tajahuerce, M., Benavent, M., Cifrian, M., Falcon, A., Fonfria, M., Del Olmo, M., Reboll, R., and Conde, A. (2018). Successful second-line metronomic temozolomide in metastatic paraganglioma: Case reports and review of the literature. Clin. Med. Insights Oncol., 12.","DOI":"10.1177\/1179554918763367"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/S0079-6603(08)60879-X","article-title":"Structure, function, and inhibition of O6-alkylguanine-DNA alkyltransferase","volume":"Volume 51","author":"Pegg","year":"1995","journal-title":"Progress in Nucleic Acid Research and Molecular Biology"},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"4189","DOI":"10.1200\/JCO.2007.11.5964","article-title":"Correlation of O6-methylguanine methyltransferase (MGMT) promoter methylation with clinical outcomes in glioblastoma and clinical strategies to modulate MGMT activity","volume":"26","author":"Hegi","year":"2008","journal-title":"J. Clin. Oncol."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/S1383-5742(00)00016-8","article-title":"Unmasking a killer: DNA O6-methylguanine and the cytotoxicity of methylating agents","volume":"462","author":"Bignami","year":"2000","journal-title":"Mutat. Res."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"e26471","DOI":"10.1002\/pbc.26471","article-title":"Vincristine-induced peripheral neuropathy in survivors of childhood acute lymphoblastic leukaemia","volume":"64","author":"Tay","year":"2017","journal-title":"Pediatric Blood Cancer"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"4040","DOI":"10.1210\/jc.2012-2356","article-title":"Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas","volume":"97","author":"Chougnet","year":"2012","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"1113","DOI":"10.1038\/s41416-019-0474-x","article-title":"A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: The SNIPP trial","volume":"120","author":"Ezzat","year":"2019","journal-title":"Br. J. Cancer"},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1200\/JCO.2016.70.7398","article-title":"Cabozantinib versus sunitinib as initial targeted therapy for patients with metastatic renal cell carcinoma of poor or intermediate risk: The Alliance A031203 CABOSUN trial","volume":"35","author":"Choueiri","year":"2017","journal-title":"J. Clin. Oncol."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.ejca.2018.02.012","article-title":"Cabozantinib versus sunitinib as initial therapy for metastatic renal cell carcinoma of intermediate or poor risk (Alliance A031203 CABOSUN randomised trial): Progression-free survival by independent review and overall survival update","volume":"94","author":"Choueiri","year":"2018","journal-title":"Eur. J. Cancer"},{"key":"ref_159","doi-asserted-by":"crossref","unstructured":"Fankhauser, M., Bechmann, N., Lauseker, M., Goncalves, J., Favier, J., Klink, B., William, D., Gieldon, L., Maurer, J., and Spottl, G. (2019). Synergistic highly potent targeted drug combinations in different pheochromocytoma models including human tumor cultures. Endocrinology.","DOI":"10.1210\/en.2019-00410"},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1007\/s11912-017-0643-0","article-title":"Treatment for malignant pheochromocytomas and paragangliomas: 5 years of progress","volume":"19","author":"Jimenez","year":"2017","journal-title":"Curr. Oncol. Rep."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1007\/s12020-017-1359-5","article-title":"Phase II trial of pazopanib in advanced\/progressive malignant pheochromocytoma and paraganglioma","volume":"57","author":"Jasim","year":"2017","journal-title":"Endocrine"},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"6162","DOI":"10.1002\/cncr.27675","article-title":"Phase 2 study of everolimus monotherapy in patients with nonfunctioning neuroendocrine tumors or pheochromocytomas\/paragangliomas","volume":"118","author":"Oh","year":"2012","journal-title":"Cancer"},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1055\/s-0029-1220687","article-title":"Novel and evolving therapies in the treatment of malignant phaeochromocytoma: Experience with the mTOR inhibitor everolimus (RAD001)","volume":"41","author":"Druce","year":"2009","journal-title":"Horm. Metab. Res."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1530\/JME-12-0028","article-title":"Combined blockade of signalling pathways shows marked anti-tumour potential in phaeochromocytoma cell lines","volume":"49","author":"Garcia","year":"2012","journal-title":"J. Mol. Endocrinol."},{"key":"ref_165","doi-asserted-by":"crossref","unstructured":"N\u00f6lting, S., Giubellino, A., Tayem, Y., Young, K., Lauseker, M., Bullova, P., Schovanek, J., Anver, M., Fliedner, S., and Korbonits, M. (2014). Combination of 13-Cis retinoic acid and lovastatin: Marked anti-tumor potential in vivo in a pheochromocytoma allograft model in female athymic nude mice. Endocrinology.","DOI":"10.1210\/en.2014-1027"},{"key":"ref_166","doi-asserted-by":"crossref","unstructured":"N\u00f6lting, S., Maurer, J., Spottl, G., Aristizabal Prada, E.T., Reuther, C., Young, K., Korbonits, M., Goke, B., Grossman, A., and Auernhammer, C.J. (2015). Additive anti-tumor effects of lovastatin and everolimus in vitro through simultaneous inhibition of signaling pathways. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0143830"},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1210\/en.2012-1854","article-title":"Combined inhibition of mTORC1 and mTORC2 signaling pathways is a promising therapeutic option in inhibiting pheochromocytoma tumor growth: In vitro and in vivo studies in female athymic nude mice","volume":"154","author":"Giubellino","year":"2013","journal-title":"Endocrinology"},{"key":"ref_168","doi-asserted-by":"crossref","unstructured":"Pang, Y., Liu, Y., Pacak, K., and Yang, C. (2019). Pheochromocytomas and paragangliomas: From genetic diversity to targeted therapies. Cancers, 11.","DOI":"10.3390\/cancers11040436"},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.coph.2016.06.009","article-title":"A2A adenosine receptor antagonists to weaken the hypoxia-HIF-1alpha driven immunosuppression and improve immunotherapies of cancer","volume":"29","author":"Hatfield","year":"2016","journal-title":"Curr. Opin. Pharmacol."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"e1062967","DOI":"10.1080\/2162402X.2015.1062967","article-title":"Hypoxia-induced soluble CD137 in malignant cells blocks CD137L-costimulation as an immune escape mechanism","volume":"5","author":"Labiano","year":"2016","journal-title":"Oncoimmunology"},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1038\/onc.2016.225","article-title":"Hypoxic stress: Obstacles and opportunities for innovative immunotherapy of cancer","volume":"36","author":"Chouaib","year":"2017","journal-title":"Oncogene"}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/11\/10\/1505\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:28:21Z","timestamp":1760189301000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/11\/10\/1505"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,8]]},"references-count":171,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2019,10]]}},"alternative-id":["cancers11101505"],"URL":"https:\/\/doi.org\/10.3390\/cancers11101505","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,8]]}}}