{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T23:42:50Z","timestamp":1776469370079,"version":"3.51.2"},"reference-count":70,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Metab Res"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pheochromocytoma and paraganglioma are rare neuroendocrine tumors with a\n                    prevalence of less than 0.05%. Being rather uncommon, they pose significant\n                    diagnostic challenges as the symptom complex is nonspecific. We present a\n                    clinical review based on the published literature and our center\u2019s experience in\n                    managing pheochromocytoma and paragangliomas over the past two decades. At the\n                    onset, summaries of three representative pheochromocytoma and paraganglioma\n                    cases are provided to highlight common diagnostic challenges. A holistic\n                    approach, combining biochemical evaluation with particular attention to pretest\n                    probability, accurate interpretation of imaging data, and differentiation from\n                    other adrenal masses, can aid in establishing the diagnosis and guiding\n                    appropriate management. Thus, the careful selection and interpretation of\n                    clinical, biochemical and imaging parameters are essential to improve diagnostic\n                    accuracy and optimize patient management.<\/jats:p>","DOI":"10.1055\/a-2792-9643","type":"journal-article","created":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T22:56:02Z","timestamp":1775775362000},"page":"125-135","source":"Crossref","is-referenced-by-count":0,"title":["Diagnostic and Management Challenges in Pheochromocytoma and\n                    Paraganglioma: A Clinical Review"],"prefix":"10.1055","volume":"58","author":[{"given":"Aditya","family":"Phadte","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manjiri","family":"Karlekar","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vijaya","family":"Sarathi","sequence":"additional","affiliation":[{"name":"Endocrinology, Vydehi Institute of Medical Sciences and\n                    Research Centre, Bengaluru, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anurag","family":"Lila","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saba Samad","family":"Memon","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rohit","family":"Barnabas","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nalini","family":"Shah","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6902-4639","authenticated-orcid":false,"given":"Tushar","family":"Bandgar","sequence":"additional","affiliation":[{"name":"Endocrinology, Seth GS Medical College and KEM Hospital,\n                    Mumbai, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2026,4,9]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1007\/s12022-022-09704-6","article-title":"Overview of the 2022 WHO classification of paragangliomas and\n                    pheochromocytomas","volume":"33","author":"O Mete","year":"2022","journal-title":"Endocr 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Metab"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"1122","DOI":"10.1210\/jc.2016-3829","article-title":"Characteristics of pediatric vs adult pheochromocytomas and paragangliomas","volume":"102","author":"C Pamporaki","year":"2017","journal-title":"J Clin Endocrinol Metab"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1530\/EJE-16-0126","article-title":"Germline mutations and genotype-phenotype correlation in Asian Indian patients\n                    with pheochromocytoma and paraganglioma","volume":"175","author":"R Pandit","year":"2016","journal-title":"Eur J Endocrinol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"e1917","DOI":"10.1210\/clinem\/dgac014","article-title":"Universal germline panel testing for individuals with pheochromocytoma and\n                    paraganglioma produces high diagnostic yield","volume":"107","author":"C Horton","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1007\/s12098-009-0287-7","article-title":"Pheochromocytoma in children and adolescents","volume":"76","author":"H K Ganesh","year":"2009","journal-title":"Indian J Pediatr"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"759","DOI":"10.4158\/EP11058.OR","article-title":"Performance of plasma fractionated free metanephrines by enzyme immunoassay in\n                    the diagnosis of pheochromocytoma and paraganglioma","volume":"17","author":"V Sarathi","year":"2011","journal-title":"Endocr Pract"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"694","DOI":"10.4158\/EP12050.OR","article-title":"Performance of plasma fractionated free metanephrines by enzyme immunoassay in\n                    the diagnosis of pheochromocytoma and paraganglioma in children","volume":"18","author":"V Sarathi","year":"2012","journal-title":"Endocr Pract"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1530\/EC-16-0086","article-title":"Predictors of malignancy in patients with pheochromocytomas\/paragangliomas:\n                    Asian Indian experience","volume":"5","author":"K Khadilkar","year":"2016","journal-title":"Endocr Connect"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1515\/jpem-2016-0375","article-title":"Genotype-phenotype correlation in paediatric pheochromocytoma and paraganglioma:\n                    A single centre experience from India","volume":"30","author":"K Khadilkar","year":"2017","journal-title":"J Pediatr Endocrinol Metab"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"745","DOI":"10.1111\/1754-9485.12620","article-title":"Genetic status determines 18 F-FDG uptake in pheochromocytoma\/paraganglioma","volume":"61","author":"A Tiwari","year":"2017","journal-title":"J Med Imaging Radiat 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Review of a 50-year\n                    autopsy series","volume":"56","author":"M G Sutton","year":"1981","journal-title":"Mayo Clin Proc"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"556","DOI":"10.1016\/j.ejso.2004.03.006","article-title":"Mortality associated with pheochromocytoma in a large Swedish cohort","volume":"30","author":"A Khorram-Manesh","year":"2004","journal-title":"Eur J Surg Oncol"},{"key":"ref31","doi-asserted-by":"publisher","first-page":"894","DOI":"10.1016\/S2213-8587(20)30314-4","article-title":"Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: A\n                    population-based cohort study","volume":"8","author":"A Ebbehoj","year":"2020","journal-title":"Lancet Diabetes Endocrinol"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1210\/jcem.85.2.6372","article-title":"A survey on adrenal incidentaloma in Italy. Study group on adrenal tumors of the\n                    Italian Society of Endocrinology","volume":"85","author":"F Mantero","year":"2000","journal-title":"J Clin Endocrinol Metab"},{"key":"ref33","doi-asserted-by":"publisher","first-page":"1872","DOI":"10.3390\/ijerph16101872","article-title":"Malignancy risk and hormonal activity of adrenal incidentalomas in a large\n                    cohort of patients from a single tertiary reference center","volume":"16","author":"E Cyranska-Chyrek","year":"2019","journal-title":"Int J Environ Res Public Health"},{"key":"ref34","doi-asserted-by":"publisher","first-page":"e389","DOI":"10.1210\/clinem\/dgad401","article-title":"Pheochromocytomas most commonly present as adrenal incidentalomas: A large\n                    tertiary center experience","volume":"109","author":"S Aggarwal","year":"2023","journal-title":"J Clin Endocrinol Metab"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.1177\/20420188231207544","article-title":"Pheochromocytoma: A changing perspective and current concepts","volume":"14","author":"A Kiriakopoulos","year":"2023","journal-title":"Ther Adv Endocrinol Metab"},{"key":"ref36","doi-asserted-by":"publisher","first-page":"1427","DOI":"10.1001\/jama.287.11.1427","article-title":"Biochemical diagnosis of pheochromocytoma: Which test is best?","volume":"287","author":"JW M Lenders","year":"2002","journal-title":"JAMA"},{"key":"ref37","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1210\/endrev\/bnad011","article-title":"Biochemical assessment of pheochromocytoma and paraganglioma","volume":"44","author":"G Eisenhofer","year":"2023","journal-title":"Endocr Rev"},{"key":"ref38","doi-asserted-by":"publisher","first-page":"1646","DOI":"10.1373\/clinchem.2018.291369","article-title":"Biochemical diagnosis of chromaffin cell tumors in patients at high and low risk\n                    of disease: Plasma versus urinary free or deconjugated O-methylated\n                    catecholamine metabolites","volume":"64","author":"G Eisenhofer","year":"2018","journal-title":"Clin Chem"},{"key":"ref39","doi-asserted-by":"publisher","first-page":"478","DOI":"10.1111\/cen.12327","article-title":"Biochemical diagnosis of phaeochromocytoma using plasma-free normetanephrine,\n                    metanephrine and methoxytyramine: Importance of supine sampling under fasting\n                    conditions","volume":"80","author":"R D\u00e4rr","year":"2014","journal-title":"Clin Endocrinol (Oxf)"},{"key":"ref40","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1016\/j.cca.2018.12.019","article-title":"Reference intervals for LC-MS\/MS measurements of plasma free, urinary free and\n                    urinary acid-hydrolyzed deconjugated normetanephrine, metanephrine and\n                    methoxytyramine","volume":"490","author":"G Eisenhofer","year":"2019","journal-title":"Clin Chim Acta"},{"key":"ref41","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1530\/EJE-14-0730","article-title":"Measurements of plasma metanephrines by immunoassay vs liquid chromatography\n                    with tandem mass spectrometry for diagnosis of pheochromocytoma","volume":"172","author":"D Weismann","year":"2015","journal-title":"Eur J Endocrinol"},{"key":"ref42","doi-asserted-by":"publisher","first-page":"1021420","DOI":"10.3389\/fendo.2022.1021420","article-title":"Silent pheochromocytoma and paraganglioma: Systematic review and proposed\n                    definitions for standardized terminology","volume":"13","author":"G Constantinescu","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"key":"ref43","doi-asserted-by":"publisher","first-page":"e28081","DOI":"10.1002\/pbc.28081","article-title":"Biochemical testing for neuroblastoma using plasma free 3-O-methyldopa,\n                    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pregnancy","volume":"24","author":"R J Clifton-Bligh","year":"2023","journal-title":"Rev Endocr Metab Disord"},{"key":"ref47","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1210\/endrev\/bnae001","article-title":"Imaging of pheochromocytomas and paragangliomas","volume":"45","author":"Ta\u00efeb D Timmers HJLM","year":"2024","journal-title":"Endocr Rev"},{"key":"ref48","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1530\/EJE-18-0006","article-title":"Unenhanced CT imaging is highly sensitive to exclude pheochromocytoma: A\n                    multicenter study","volume":"178","author":"E Buitenwerf","year":"2018","journal-title":"Eur J Endocrinol"},{"key":"ref49","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1210\/jc.2018-01532","article-title":"CT characteristics of pheochromocytoma: Relevance for the evaluation of adrenal\n                    incidentaloma","volume":"104","author":"L Canu","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"key":"ref50","doi-asserted-by":"publisher","first-page":"2040","DOI":"10.1016\/j.mayocp.2019.03.030","article-title":"Diagnostic accuracy of computed tomography to exclude pheochromocytoma: A\n                    systematic review, meta-analysis, and cost analysis","volume":"94","author":"E Buitenwerf","year":"2019","journal-title":"Mayo Clin Proc"},{"key":"ref51","doi-asserted-by":"publisher","first-page":"G1","DOI":"10.1093\/ejendo\/lvad066","article-title":"European Society of Endocrinology clinical practice guidelines on the management\n                    of adrenal incidentalomas, in collaboration with the European Network for the\n                    Study of Adrenal Tumors","volume":"189","author":"M Fassnacht","year":"2023","journal-title":"Eur J Endocrinol"},{"key":"ref52","doi-asserted-by":"publisher","first-page":"1027","DOI":"10.1007\/s00330-017-5076-5","article-title":"Pheochromocytoma as a frequent false-positive in adrenal washout CT: A\n                    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N\u00f6lting","year":"2022","journal-title":"Endocr Rev"},{"key":"ref56","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1016\/s0009-9260(97)80252-8","article-title":"MR differentiation of phaeochromocytoma from other adrenal lesions based on\n                    qualitative analysis of T2 relaxation times","volume":"52","author":"J C Varghese","year":"1997","journal-title":"Clin Radiol"},{"key":"ref57","doi-asserted-by":"publisher","first-page":"2885","DOI":"10.1007\/s00330-008-1073-z","article-title":"Adrenal phaeochromocytoma: Correlation of MRI appearances with histology and\n                    function","volume":"18","author":"AE T Jacques","year":"2008","journal-title":"Eur Radiol"},{"key":"ref58","doi-asserted-by":"publisher","first-page":"825","DOI":"10.2214\/AJR.12.9576","article-title":"Multimodality imaging findings of pheochromocytoma with associated clinical and\n                    biochemical features in 53 patients with histologically confirmed tumors","volume":"201","author":"A Raja","year":"2013","journal-title":"AJR Am J Roentgenol"},{"key":"ref59","doi-asserted-by":"publisher","first-page":"2112","DOI":"10.1007\/s00259-019-04398-1","article-title":"European association of nuclear medicine practice guideline\/society of nuclear\n                    medicine and molecular imaging procedure standard 2019 for radionuclide imaging\n                    of phaeochromocytoma and paraganglioma","volume":"46","author":"D Ta\u00efeb","year":"2019","journal-title":"Eur J Nucl Med Mol Imaging"},{"key":"ref60","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1210\/clinem\/dgae764","article-title":"Positron emission tomography imaging of pheochromocytoma and\n                    paraganglioma-18F-FDOPA vs somatostatin analogues","volume":"110","author":"A Ebbehoj","year":"2025","journal-title":"J Clin Endocrinol Metab"},{"key":"ref61","doi-asserted-by":"publisher","first-page":"1459","DOI":"10.1056\/NEJMoa020152","article-title":"Germline mutations in nonsyndromic pheochromocytoma","volume":"346","author":"HP H Neumann","year":"2002","journal-title":"N Engl J Med"},{"key":"ref62","doi-asserted-by":"publisher","first-page":"470","DOI":"10.4103\/ijem.IJEM_558_16","article-title":"Characteristics of pediatric pheochromocytoma\/paraganglioma","volume":"21","author":"V Sarathi","year":"2017","journal-title":"Indian J Endocrinol Metab"},{"key":"ref63","doi-asserted-by":"publisher","first-page":"1915","DOI":"10.1210\/jc.2014-1498","article-title":"Pheochromocytoma and paraganglioma: An endocrine society clinical practice\n                    guideline","volume":"99","author":"JW M Lenders","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"ref64","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1097\/MED.0000000000000634","article-title":"Genetics of pheochromocytoma and paraganglioma","volume":"28","author":"H Wachtel","year":"2021","journal-title":"Curr Opin Endocrinol Diabetes Obes"},{"key":"ref65","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1016\/S2213-8587(23)00038-4","article-title":"Clinical consensus guideline on the management of phaeochromocytoma and\n                    paraganglioma in patients harbouring germline SDHD pathogenic variants","volume":"11","author":"D Ta\u00efeb","year":"2023","journal-title":"Lancet Diabetes Endocrinol"},{"key":"ref66","doi-asserted-by":"publisher","first-page":"101951","DOI":"10.1016\/j.beem.2024.101951","article-title":"Head and neck paragangliomas: Recent advances in translational and clinical\n                    research and guidelines for patient care","volume":"38","author":"S Richter","year":"2024","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"key":"ref67","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1210\/clinem\/dgac657","article-title":"Efficacy and safety 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