{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T17:44:53Z","timestamp":1790790293449,"version":"4.1.0"},"reference-count":10,"publisher":"The Endocrine Society","issue":"3","license":[{"start":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T00:00:00Z","timestamp":1686096000000},"content-version":"vor","delay-in-days":37,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,5,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla chromaffin cells, usually associated with features of catecholamine excess. Clinically and biochemically silent pheochromocytoma without adrenergic symptoms or elevated catecholamine concentrations are rare. A 71-year-old female presented with acute right flank pain with abdominal computed tomography (CT) scan revealing a hemorrhagic right adrenal mass. She had no preceding adrenergic symptoms, and normal serum electrolytes, on a background of well-controlled hypertension on amlodipine monotherapy. After conservative management and discharge, an outpatient CT adrenal scan confirmed an 88 \u00d7 64\u2005mm right adrenal mass demonstrating intense avidity (maximum standardized uptake value, 20.2) on fluorodeoxyglucose F 18-positron emission tomography (FDG-PET)\/CT scan. Biochemical screening supported a nonfunctional adrenal lesion with normal-range plasma normetanephrines and metanephrines. She underwent a right adrenalectomy for presumed nonfunctioning adrenocortical cancer; however, histopathology demonstrated a 120-mm pheochromocytoma. Succinate dehydrogenase subunit B (SDHB) and fumarate hydratase (FH) staining were retained; however, weakly positive 2SC staining raised concerns for FH-deficient pheochromocytoma. Germline DNA sequencing was negative for pathogenic RET, VHL, SDHB, SDHD, or FH variants. Tumor cells stained positive for tyrosine hydroxylase and negative for dopamine \u03b2 hydroxylase. Four months postoperatively, progress FDG-PET\/CT scan demonstrated no focal avidity. Massive biochemically silent pheochromocytomas are exceedingly rare, and we discuss various mechanisms that may predispose patients to this phenomenon.<\/jats:p>","DOI":"10.1210\/jcemcr\/luad061","type":"journal-article","created":{"date-parts":[[2023,6,9]],"date-time":"2023-06-09T16:59:23Z","timestamp":1686329963000},"source":"Crossref","is-referenced-by-count":2,"title":["Massive Biochemically Silent Pheochromocytoma Masquerading as Nonfunctioning Adrenocortical Cancer"],"prefix":"10.1210","volume":"1","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1242-2476","authenticated-orcid":false,"given":"Shejil","family":"Kumar","sequence":"first","affiliation":[{"name":"Department of Diabetes, Endocrinology & Metabolism, Royal North Shore Hospital , Sydney, NSW 2065 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Diabetes, Endocrinology & Metabolism, Royal North Shore Hospital , Sydney, NSW 2065 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natassia","family":"Rodrigo","sequence":"additional","affiliation":[{"name":"Department of Diabetes, Endocrinology & Metabolism, Royal North Shore Hospital , Sydney, NSW 2065 , Australia"},{"name":"Northern Sydney Clinical School, Faculty of Medicine, University of Sydney , Sydney, NSW 2065 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0762-0977","authenticated-orcid":false,"given":"Anthony","family":"Glover","sequence":"additional","affiliation":[{"name":"Endocrine Surgical Unit, Royal North Shore Hospital & Northern Clinical School, Sydney, Medical School, Faculty of Medicine and Health, University of Sydney , Sydney, NSW 2065 , Australia"},{"name":"The Kinghorn Cancer Centre, Garvan Institute of Medical Research, St. Vincent's Clinical School, Faculty of Medicine, University of New South Wales , Darlinghurst, NSW 2010 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2023,6,7]]},"reference":[{"issue":"1","key":"2023060915171460700_luad061-B1","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1586\/eem.12.76","article-title":"Diagnosis of silent pheochromocytoma and paraganglioma","volume":"8","author":"Clifton-Bligh","year":"2013","journal-title":"Expert Rev Endocrinol Metab"},{"key":"2023060915171460700_luad061-B2","doi-asserted-by":"crossref","first-page":"1021420","DOI":"10.3389\/fendo.2022.1021420","article-title":"Silent pheochromocytoma and paraganglioma: systematic review and proposed definitions for standardized terminology","volume":"13","author":"Constantinescu","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"issue":"1","key":"2023060915171460700_luad061-B3","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/s00268-011-1338-6","article-title":"Spontaneous adrenal hemorrhage with associated masses: etiology and management in 6 cases and a review of 133 reported cases","volume":"36","author":"Marti","year":"2012","journal-title":"World J Surg"},{"issue":"4","key":"2023060915171460700_luad061-B4","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1210\/clinem\/dgac672","article-title":"Approach to the patient with adrenal hemorrhage","volume":"108","author":"Elhassan","year":"2023;","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2023060915171460700_luad061-B5","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1515\/cclm-2020-0904","article-title":"Plasma metanephrines and prospective prediction of tumor location, size and mutation type in patients with pheochromocytoma and paraganglioma","volume":"59","author":"Eisenhofer","year":"2020","journal-title":"Clin Chem Lab Med"},{"issue":"1","key":"2023060915171460700_luad061-B6","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1002\/ccr3.149","article-title":"A giant cystic pheochromocytoma mimicking liver abscess an unusual presentation - a case report","volume":"3","author":"Sarveswaran","year":"2015","journal-title":"Clin Case Rep"},{"issue":"9","key":"2023060915171460700_luad061-B7","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2019-230730","article-title":"Simple adrenal cyst masquerading clinically silent giant cystic pheochromocytoma","volume":"12","author":"Kumar","year":"2019","journal-title":"BMJ Case Rep"},{"issue":"8","key":"2023060915171460700_luad061-B8","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.3390\/cancers11081121","article-title":"Intricacies of the molecular machinery of catecholamine biosynthesis and secretion by chromaffin cells of the normal adrenal medulla and in pheochromocytoma and paraganglioma","volume":"11","author":"Berends","year":"2019","journal-title":"Cancers (Basel)"},{"issue":"12","key":"2023060915171460700_luad061-B9","doi-asserted-by":"crossref","DOI":"10.1210\/jendso\/bvab163","article-title":"Dopamine-secreting pheochromocytoma and paraganglioma","volume":"5","author":"Miyamoto","year":"2021","journal-title":"J Endocr Soc"},{"issue":"6","key":"2023060915171460700_luad061-B10","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"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/1\/3\/luad061\/50526278\/luad061.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/1\/3\/luad061\/50526278\/luad061.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,6,9]],"date-time":"2023-06-09T16:59:30Z","timestamp":1686329970000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luad061\/7191292"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5]]},"references-count":10,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,5,8]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luad061","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,5]]},"published":{"date-parts":[[2023,5]]},"article-number":"luad061"}}