Outcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centers
dc.contributor.author | Fountas, A | |
dc.contributor.author | Lim, ES | |
dc.contributor.author | Drake, WM | |
dc.contributor.author | Powlson, AS | |
dc.contributor.author | Gurnell, M | |
dc.contributor.author | Martin, NM | |
dc.contributor.author | Seejore, K | |
dc.contributor.author | Murray, RD | |
dc.contributor.author | MacFarlane, J | |
dc.contributor.author | Ahluwalia, R | |
dc.contributor.author | Swords, F | |
dc.contributor.author | Ashraf, M | |
dc.contributor.author | Pal, A | |
dc.contributor.author | Chong, Z | |
dc.contributor.author | Freel, M | |
dc.contributor.author | Balafshan, T | |
dc.contributor.author | Purewal, TS | |
dc.contributor.author | Speak, RG | |
dc.contributor.author | Newell-Price, J | |
dc.contributor.author | Higham, Claire E | |
dc.contributor.author | Hussein, Z | |
dc.contributor.author | Baldeweg, SE | |
dc.contributor.author | Dales, J | |
dc.contributor.author | Reddy, N | |
dc.contributor.author | Levy, MJ | |
dc.contributor.author | Karavitaki, N | |
dc.date.accessioned | 2019-12-09T17:23:35Z | |
dc.date.available | 2019-12-09T17:23:35Z | |
dc.date.issued | 2019 | en |
dc.identifier.citation | Fountas 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.pmid | 31735971 | en |
dc.identifier.doi | 10.1210/clinem/dgz200 | en |
dc.identifier.uri | http://hdl.handle.net/10541/622617 | |
dc.description.abstract | CONTEXT: 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.iso | en | en |
dc.relation.url | https://dx.doi.org/10.1210/clinem/dgz200 | en |
dc.title | Outcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centers | en |
dc.type | Article | en |
dc.contributor.department | Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK | en |
dc.identifier.journal | Journal of Clinical Endocrinology and Metabolism | en |