{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:48Z","timestamp":1784557068921,"version":"3.55.0"},"reference-count":10,"publisher":"Bioscientifica","issue":"3","license":[{"start":{"date-parts":[[2023,7,1]],"date-time":"2023-07-01T00:00:00Z","timestamp":1688169600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,7,1]]},"abstract":"<jats:sec>\n                    <jats:title>Summary<\/jats:title>\n                    <jats:p>\n                      This case report describes a rare presentation of ectopic Cushing\u2019s syndrome (CS) due to ectopic corticotropin-releasing hormone (CRH) production from a medullary thyroid carcinoma (MTC). The patient, a 69-year-old man, presented with symptoms of muscle weakness, facial plethora, and easy bruising. An inferior petrosal sinus sampling test (IPSS) demonstrated pituitary adrenocorticotrophic hormone (ACTH) secretion, but a whole-body somatostatin receptor scintigraphy (\n                      <jats:sup>68<\/jats:sup>\n                      Ga-DOTATOC PET\/CT) revealed enhanced uptake in the right thyroid lobe which, in addition to a grossly elevated serum calcitonin level, was indicative of an MTC. A\n                      <jats:sup>18<\/jats:sup>\n                      F-DOPA PET\/CT scan supported the diagnosis, and histology confirmed the presence of MTC with perinodal growth and regional lymph node metastasis. On immunohistochemical analysis, the tumor cell stained positively for calcitonin and CRH but negatively for ACTH. Distinctly elevated plasma CRH levels were documented. The patient therefore underwent thyroidectomy and bilateral adrenalectomy. This case shows that CS caused by ectopic CRH secretion may masquerade as CS due to a false positive IPSS test. It also highlights the importance of considering rare causes of CS when diagnostic test results are ambiguous.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Learning points<\/jats:title>\n                    <jats:list list-type=\"bullet\">\n                      <jats:list-item>\n                        <jats:p>Medullary thyroid carcinoma may secrete CRH and cause ectopic CS.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Ectopic CRH secretion entails a rare pitfall of inferior petrosal sinus sampling yielding a false positive test.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Plasma CRH measurements can be useful in selected cases.<\/jats:p>\n                      <\/jats:list-item>\n                    <\/jats:list>\n                  <\/jats:sec>","DOI":"10.1530\/edm-23-0057","type":"journal-article","created":{"date-parts":[[2023,9,28]],"date-time":"2023-09-28T05:13:50Z","timestamp":1695878030000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":2,"title":["Ectopic Cushing\u2019s syndrome from a corticotropin-releasing hormone-secreting medullary thyroid carcinoma: a rare pitfall of inferior petrosal sinus sampling"],"prefix":"10.1530","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8557-6396","authenticated-orcid":true,"given":"Vivi-Nelli","family":"M\u00e4kinen","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark"},{"name":"Department of Internal Medicine, Regional Hospital, Horsens, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stine","family":"Horsk\u00e6r Madsen","sequence":"additional","affiliation":[{"name":"Department of Pathology, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette","family":"Ji Riis-Vestergaard","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark"},{"name":"Department of Internal Medicine, G\u00f8dstrup Hospital, Herning,Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette","family":"Bjerre","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steen","family":"B\u00f8nl\u00f8kke Pedersen","sequence":"additional","affiliation":[{"name":"Steno Diabetes Center Aarhus, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvia L","family":"Asa","sequence":"additional","affiliation":[{"name":"Department of Pathology, University Health Network, Toronto,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Rolighed","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens Otto","family":"Lunde J\u00f8rgensen","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark"},{"name":"Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4805-6065","authenticated-orcid":true,"given":"Marie Juul","family":"Ornstrup","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Journal of Physiology","first-page":"493","article-title":"Cushing's syndrome: from physiological principles to diagnosis and clinical care","volume":"593","author":"Raff","year":"2015","unstructured":"Raff H & Carroll T. 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Clinical Epidemiology20157281\u2013293. (https:\/\/doi.org\/10.2147\/CLEP.S44336)"},{"key":"bib10","series-title":"Journal of the Endocrine Society","first-page":"bvac073","article-title":"The Mount Sinai clinical pathway for the diagnosis and management of hypercortisolism due to ectopic ACTH syndrome","volume":"6","author":"Alba","year":"2022","unstructured":"Alba ELJapp EAFernandez-Ranvier GBadani KWilck EGhesani MWolf AWolin EMCorbett VSteinmetz D, The Mount Sinai clinical pathway for the diagnosis and management of hypercortisolism due to ectopic ACTH syndrome. Journal of the Endocrine Society20226bvac073. 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