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dc.contributor.authorFountas, A
dc.contributor.authorLim, ES
dc.contributor.authorDrake, WM
dc.contributor.authorPowlson, AS
dc.contributor.authorGurnell, M
dc.contributor.authorMartin, NM
dc.contributor.authorSeejore, K
dc.contributor.authorMurray, RD
dc.contributor.authorMacFarlane, J
dc.contributor.authorAhluwalia, R
dc.contributor.authorSwords, F
dc.contributor.authorAshraf, M
dc.contributor.authorPal, A
dc.contributor.authorChong, Z
dc.contributor.authorFreel, M
dc.contributor.authorBalafshan, T
dc.contributor.authorPurewal, TS
dc.contributor.authorSpeak, RG
dc.contributor.authorNewell-Price, J
dc.contributor.authorHigham, Claire E
dc.contributor.authorHussein, Z
dc.contributor.authorBaldeweg, SE
dc.contributor.authorDales, J
dc.contributor.authorReddy, N
dc.contributor.authorLevy, MJ
dc.contributor.authorKaravitaki, N
dc.date.accessioned2019-12-09T17:23:35Z
dc.date.available2019-12-09T17:23:35Z
dc.date.issued2019en
dc.identifier.citationFountas A, Lim ES, Drake WM, Powlson AS, Gurnell M, Martin NM, et al. Outcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centers. J Clin Endocrinol Metab. 2019.en
dc.identifier.pmid31735971en
dc.identifier.doi10.1210/clinem/dgz200en
dc.identifier.urihttp://hdl.handle.net/10541/622617
dc.description.abstractCONTEXT: Long-term outcomes of patients with Nelson's syndrome (NS) have been poorly explored, especially in the modern era. OBJECTIVE: To elucidate tumor control rates, effectiveness of various treatments and markers of prognostic relevance in patients with NS. PATIENTS, DESIGN, AND SETTING: Retrospective cohort study of 68 patients from 13 UK pituitary centers with median imaging follow-up of 13 years (range 1-45) since NS diagnosis. RESULTS: Management of Cushing's disease (CD) prior to NS diagnosis included surgery+adrenalectomy (n=30, eight patients had two and one had three pituitary operations), surgery+radiotherapy+adrenalectomy (n=17, two received >1 courses of irradiation, two had ?2 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, 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 (HR 4.6; 95% CI, 1.6-13.5). During follow-up, 3% of patients had malignant transformation with spinal metastases and 4% died of aggressively enlarging tumor. CONCLUSIONS: 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.en
dc.language.isoenen
dc.relation.urlhttps://dx.doi.org/10.1210/clinem/dgz200en
dc.titleOutcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centersen
dc.typeArticleen
dc.contributor.departmentInstitute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UKen
dc.identifier.journalJournal of Clinical Endocrinology and Metabolismen


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