{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T07:01:57Z","timestamp":1791874917263,"version":"4.3.4"},"reference-count":45,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,11,20]],"date-time":"2015-11-20T00:00:00Z","timestamp":1447977600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2016,2]]},"abstract":"Summary<\/jats:title>Introduction<\/jats:title>10% of corticotrophin (ACTH<\/jats:styled-content>)\u2010dependent Cushing's syndrome arises from secretion by extrapituitary tumours, with phaeochromocytoma implicated in a few cases. Ectopic secretion by phaeochromocytoma of corticotropin\u2010releasing hormone (CRF<\/jats:styled-content>), with secondary corticotroph hyperplasia, is even rarer, with only five cases in the literature hitherto. However, such cases may be classified as \u2018ectopic ACTH<\/jats:styled-content>\u2019 due to incomplete verification.<\/jats:p><\/jats:sec>Clinical cases<\/jats:title>We describe three patients with phaeochromocytoma and ACTH<\/jats:styled-content>\u2010dependent Cushing's syndrome in whom biochemical cure was achieved following unilateral adrenalectomy. Although unable to access a validated CRF<\/jats:styled-content> assay within the timeframe for sample storage, we nevertheless inferred CRF<\/jats:styled-content> secretion in 2 of 3 cases by tumour immunostaining (positive for CRF<\/jats:styled-content>; negative for ACTH<\/jats:styled-content>), supported in one case by pre\u2010operative inferior petrosal sinus sampling (IPSS<\/jats:styled-content>) indicative of pituitary ACTH<\/jats:styled-content> source. Both cases were characterized by rapid postoperative wean off glucocorticoids, presumed to reflect the pituitary stimulatory\u2010effect of CRF<\/jats:styled-content> outweighing central negative feedback inhibition by hypercortisolaemia. By contrast, the tumour excised in a third case exhibited positive immunostaining for ACTH<\/jats:styled-content> \u2013 negative for CRF<\/jats:styled-content> \u2013 and postoperative recovery of hypothalamic\u2013pituitary\u2013adrenal axis took significantly longer.<\/jats:p><\/jats:sec>Discussion<\/jats:title>Ectopic CRF<\/jats:styled-content> production is biochemically indistinguishable from ectopic ACTH<\/jats:styled-content> secretion, except that IPSS<\/jats:styled-content> mimics pituitary Cushing's disease and cortisol dynamics may normalize rapidly postadrenalectomy. CRF<\/jats:styled-content> secretion can be inferred through tumour immunohistochemistry, even if no CRF<\/jats:styled-content> assay is available. Unrecognized phaeochromocytoma ACTH<\/jats:styled-content> secretion may underpin some cases of cardiovascular collapse postadrenalectomy through acute hypocortisolaemia. Despite advances in phaeochromocytoma genetics since previous reports, we were unable to identify somatic DNA<\/jats:styled-content> defects associated with either ACTH<\/jats:styled-content> or CRF<\/jats:styled-content> secretion.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.12960","type":"journal-article","created":{"date-parts":[[2015,10,3]],"date-time":"2015-10-03T12:58:47Z","timestamp":1443877127000},"page":"177-184","source":"Crossref","is-referenced-by-count":17,"title":["Phaeochromocytoma and ACTH<\/scp>\u2010dependent cushing's syndrome: tumour crf secretion can mimic pituitary cushing's disease"],"prefix":"10.1111","volume":"84","author":[{"given":"Konstantinos B.","family":"Lois","sequence":"first","affiliation":[{"name":"Department of Endocrinology Newcastle\u2010upon\u2010Tyne Hospitals NHS Foundation Trust Newcastle\u2010upon\u2010Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anjali","family":"Santhakumar","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Newcastle\u2010upon\u2010Tyne Hospitals NHS Foundation Trust Newcastle\u2010upon\u2010Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suresh","family":"Vaikkakara","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Sri Venkateswara Institute of Medical Sciences Tirupati (MP) India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sajjan","family":"Mathew","sequence":"additional","affiliation":[{"name":"Department of Surgery Oman Health Services Sohar Hospital Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Long","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology Newcastle\u2010upon\u2010Tyne Hospitals NHS Foundation Trust Newcastle\u2010upon\u2010Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah J.","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology Newcastle\u2010upon\u2010Tyne Hospitals NHS Foundation Trust Newcastle\u2010upon\u2010Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rovert","family":"Peaston","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry Newcastle\u2010upon\u2010Tyne Hospitals NHS Foundation Trust Newcastle\u2010upon\u2010Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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