{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T14:42:11Z","timestamp":1788964931622,"version":"build-2803163510"},"reference-count":10,"publisher":"The Endocrine Society","issue":"2","license":[{"start":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T00:00:00Z","timestamp":1706313600000},"content-version":"vor","delay-in-days":19,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,1,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Medullary thyroid cancer (MTC) is a neuroendocrine tumor associated with activating mutations of the rearranged during transfection (RET) proto-oncogene. These tumors may rarely secrete adrenocorticotropin or corticotropin-releasing hormone, resulting in a paraneoplastic ectopic Cushing syndrome (ECS). Paraneoplastic ECS carries a high risk of mortality, and management is difficult due to the lack of response to antiadrenal therapies. We report on a 37-year-old man who was diagnosed with metastatic MTC and reported symptoms of cortisol excess with laboratory testing in keeping with ECS. He began treatment with vandetanib, a multitargeted tyrosine kinase inhibitor, which resulted in decreased tumor burden as well as clinical and biochemical resolution of ECS. Due to progressive structural disease 10 months later, he was switched to the selective RET inhibitor selpercatinib, which was followed by a rapid reduction of cortisol nearing the threshold of adrenal insufficiency. Tumor markers were also improved, and repeat imaging showed decreased tumor burden. Our case highlights the efficacy of tyrosine kinase inhibitors in the management of paraneoplastic ECS. Selective RET inhibitors may emerge as preferred targeted treatment options due to better efficacy and toxicity profiles compared to multitargeted inhibitors. Clinicians should monitor for adrenal insufficiency with the use of selective RET inhibitors.<\/jats:p>","DOI":"10.1210\/jcemcr\/luad174","type":"journal-article","created":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T16:05:58Z","timestamp":1706371558000},"source":"Crossref","is-referenced-by-count":7,"title":["Remission of Ectopic Cushing Syndrome Secondary to Medullary Thyroid Cancer With Vandetanib and Selpercatinib"],"prefix":"10.1210","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6478-6394","authenticated-orcid":false,"given":"Aria","family":"Jazdarehee","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Alberta , Edmonton, AB, T6G 2R7 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5117-2502","authenticated-orcid":false,"given":"Omar","family":"Abdel-Rahman","sequence":"additional","affiliation":[{"name":"Department of Oncology, Cross Cancer Institute , Edmonton, AB, T6G 1Z2 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-9775-8011","authenticated-orcid":false,"given":"Jennifer E","family":"Jacquier","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Alberta , Edmonton, AB, T6G 2R7 , Canada"},{"name":"Division of Endocrinology and Metabolism, University of Alberta , Edmonton, AB, T6G 2R7 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2024,1,27]]},"reference":[{"issue":"11","key":"2024022316561487800_luad174-B1","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1089\/thy.2021.0696","article-title":"Medullary thyroid cancer with ectopic Cushing's syndrome: a case report and systematic review of detailed cases from the literature","volume":"32","author":"Corsello","year":"2022","journal-title":"Thyroid"},{"issue":"9","key":"2024022316561487800_luad174-B2","doi-asserted-by":"crossref","first-page":"3055","DOI":"10.1210\/jc.2013-4340","article-title":"Vandetanib successfully controls medullary thyroid cancer-related cushing syndrome in an adolescent patient","volume":"99","author":"Nella","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"key":"2024022316561487800_luad174-B3","first-page":"20-0207","article-title":"Metastatic medullary thyroid carcinoma presenting as ectopic Cushing's syndrome","volume":"2021","author":"Forde","year":"2021","journal-title":"Endocrinol Diabetes Metab Case Rep"},{"issue":"6","key":"2024022316561487800_luad174-B4","doi-asserted-by":"crossref","first-page":"e220032","DOI":"10.1530\/ETJ-22-0032","article-title":"Rapid and long-lasting response to selpercatinib of paraneoplastic Cushing's syndrome in medullary thyroid 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trial","volume":"30","author":"Wells","year":"2012","journal-title":"J Clin Oncol"},{"issue":"9","key":"2024022316561487800_luad174-B8","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1056\/NEJMoa2005651","article-title":"Efficacy of selpercatinib in RET-altered thyroid cancers","volume":"383","author":"Wirth","year":"2020","journal-title":"N Engl J Med"},{"issue":"1","key":"2024022316561487800_luad174-B9","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1158\/1078-0432.CCR-20-2278","article-title":"Overcoming MET-dependent resistance to selective RET inhibition in patients with RET fusion-positive lung cancer by combining selpercatinib with crizotinib","volume":"27","author":"Rosen","year":"2021","journal-title":"Clin Cancer Res"},{"issue":"8)","key":"2024022316561487800_luad174-B10","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1007\/s40618-023-02025-3","article-title":"Adrenal insufficiency in thyroid cancer patients treated with tyrosine kinase 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