{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:45:48Z","timestamp":1790696748184,"version":"4.1.0"},"reference-count":58,"publisher":"The Endocrine Society","issue":"5","license":[{"start":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T00:00:00Z","timestamp":1574035200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Clinical Endocrinology Trust, UK"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Context<\/jats:title><jats:p>Long-term outcomes of patients with Nelson\u2019s syndrome (NS) have been poorly explored, especially in the modern era.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To elucidate tumor control rates, effectiveness of various treatments, and markers of prognostic relevance in patients with NS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients, design, and setting<\/jats:title><jats:p>Retrospective cohort study of 68 patients from 13 UK pituitary centers with median imaging follow-up of 13 years (range 1\u201345) since NS diagnosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Management of Cushing\u2019s disease (CD) prior to NS diagnosis included surgery+adrenalectomy (n = 30; eight patients had 2 and one had 3 pituitary operations), surgery+radiotherapy+adrenalectomy (n = 17; two received &amp;gt;1 courses of irradiation, two had \u22652 pituitary surgeries), radiotherapy+adrenalectomy (n = 2), and adrenalectomy (n = 19). Primary management of NS mainly included surgery, radiotherapy, surgery+radiotherapy, and observation; 10-year tumor progression-free survival was 62% (surgery 80%, radiotherapy 52%, surgery+radiotherapy 81%, observation 51%). Sex, age at CD or NS diagnosis, size of adenoma (micro-\/macroadenoma) at CD diagnosis, presence of pituitary tumor on imaging prior adrenalectomy, and mode of NS primary management were not predictors of tumor progression. Mode of management of CD before NS diagnosis was a significant factor predicting progression, with the group treated by surgery+radiotherapy+adrenalectomy for their CD showing the highest risk (hazard ratio 4.6; 95% confidence interval, 1.6\u201313.5). During follow-up, 3% of patients had malignant transformation with spinal metastases and 4% died of aggressively enlarging tumor.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>At 10 years follow-up, 38% of the patients diagnosed with NS showed progression of their corticotroph tumor. Complexity of treatments for the CD prior to NS diagnosis, possibly reflecting corticotroph adenoma aggressiveness, predicts long-term tumor prognosis.<\/jats:p><\/jats:sec>","DOI":"10.1210\/clinem\/dgz200","type":"journal-article","created":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T08:50:35Z","timestamp":1574067035000},"page":"1527-1537","source":"Crossref","is-referenced-by-count":31,"title":["Outcomes of Patients with Nelson\u2019s Syndrome after Primary Treatment: A Multicenter Study from 13 UK Pituitary Centers"],"prefix":"10.1210","volume":"105","author":[{"given":"Athanasios","family":"Fountas","sequence":"first","affiliation":[{"name":"Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK"},{"name":"Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS 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