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There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson\u2019s syndrome.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1\u00a0month pasireotide 300\u2013600\u00a0\u03bcg s.c. twice-daily; (3) 6\u00a0months pasireotide long-acting-release (LAR) 40\u201360\u00a0mg monthly.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Seven patients had s.c. treatment and 5 proceeded to LAR treatment. There was a significant reduction in morning plasma ACTH during treatment (mean\u2009\u00b1\u2009SD; 1823\u2009\u00b1\u20091286\u00a0ng\/l vs. 888.0\u2009\u00b1\u2009812.8\u00a0ng\/l during the s.c. phase vs. 829.0\u2009\u00b1\u20091171\u00a0ng\/l during the LAR phase, p\u2009&lt;\u20090.0001). Analysis of ACTH levels using a random intercept linear mixed-random effects longitudinal model showed that ACTH (before the morning dose of glucocorticoids) declined significantly by 26.1\u00a0ng\/l per week during the 28-week of treatment (95% CI \u2212\u200945.2 to \u2212\u20097.1, p\u2009&lt;\u20090.01). An acute response to a test dose predicted outcome in 4\/5 patients. Overall, there was no significant change in tumor volumes (1.4\u2009\u00b1\u20090.9 vs. 1.3\u2009\u00b1\u20091.0, p\u2009=\u20090.86). Four patients withdrew during the study. Hyperglycemia occurred in 6 patients.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Pasireotide lowers plasma ACTH levels in patients with Nelson\u2019s syndrome. A longer period of treatment may be needed to assess the effects of pasireotide on tumor volume.<\/jats:p>\n                <jats:p>Trial registration: Clinical Trials.gov ID, NCT01617733<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s11102-017-0853-3","type":"journal-article","created":{"date-parts":[[2018,1,8]],"date-time":"2018-01-08T14:44:10Z","timestamp":1515422650000},"page":"247-255","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["A prospective longitudinal study of Pasireotide in Nelson\u2019s syndrome"],"prefix":"10.1007","volume":"21","author":[{"given":"Eleni","family":"Daniel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miguel","family":"Debono","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sharon","family":"Caunt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Constantine","family":"Girio-Fragkoulakis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen J.","family":"Walters","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scott A.","family":"Akker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashley B.","family":"Grossman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter J.","family":"Trainer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Newell-Price","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2018,1,8]]},"reference":[{"key":"853_CR1","doi-asserted-by":"publisher","first-page":"560","DOI":"10.7326\/0003-4819-52-3-560","volume":"52","author":"DH Nelson","year":"1960","unstructured":"Nelson DH, Meakin JW, Thorn GW (1960) ACTH-producing pituitary tumors following adrenalectomy for Cushing\u2019s syndrome. 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The other authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed in the study involving human participants were in accordance with the ethical standards of the UK Health Research Authority (reference 10\/H1005\/53) and with the 1964 Helsinki declaration and its later amendments. This article does not contain any studies with animals performed by any of the authors.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}