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Outcomes of patients with Nelson's Syndrome after primary treatment: a multicenter study from 13 UK pituitary centers

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Title: Outcomes of patients with Nelson's Syndrome after primary treatment: a multicenter study from 13 UK pituitary centers
Authors: Fountas, A
Lim, ES
Drake, WM
Powlson, AS
Gurnell, M
Martin, NM
Seejore, K
Murray, RD
MacFarlane, J
Ahluwalia, R
Swords, F
Ashraf, M
Pal, A
Chong, Z
Freel, M
Balafshan, T
Purewal, TS
Speak, RG
Newell-Price, J
Higham, CE
Hussein, Z
Baldeweg, SE
Dales, J
Reddy, N
Levy, MJ
Karavitaki, N
Item Type: Journal Article
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 2 and one had 3 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, 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, 1.6–13.5). 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.
Issue Date: 1-May-2020
Date of Acceptance: 13-Nov-2019
URI: http://hdl.handle.net/10044/1/80631
DOI: 10.1210/clinem/dgz200
ISSN: 0021-972X
Publisher: Endocrine Society
Start Page: 1
End Page: 11
Journal / Book Title: Journal of Clinical Endocrinology and Metabolism
Volume: 105
Issue: 5
Copyright Statement: © Endocrine Society 2019. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model). This is a pre-copy-editing, author-produced version of an article accepted for publication in The Journal of Clinical Endocrinology & Metabolism following peer review. The definitive publisher-authenticated version Athanasios Fountas, Eugenie S Lim, William M Drake, Andrew S Powlson, Mark Gurnell, Niamh M Martin, Khyatisha Seejore, Robert D Murray, James MacFarlane, Rupa Ahluwalia, Francesca Swords, Muhammad Ashraf, Aparna Pal, Zhuomin Chong, Marie Freel, Tala Balafshan, Tejpal S Purewal, Rowena G Speak, John Newell-Price, Claire E Higham, Ziad Hussein, Stephanie E Baldeweg, Jolyon Dales, Narendra Reddy, Miles J Levy, Niki Karavitaki, Outcomes of Patients with Nelson’s Syndrome after Primary Treatment: A Multicenter Study from 13 UK Pituitary Centers, The Journal of Clinical Endocrinology & Metabolism, Volume 105, Issue 5, May 2020, Pages 1527–1537 is available online at: https://doi.org/10.1210/clinem/dgz200
Keywords: Science & Technology
Life Sciences & Biomedicine
Endocrinology & Metabolism
Nelson's syndrome
Cushing's
bilateral adrenalectomy
tumor progression
CUSHINGS-DISEASE
STEREOTACTIC RADIOSURGERY
BILATERAL ADRENALECTOMY
MANAGEMENT
TUMORS
EXPERIENCE
MORTALITY
ADENOMAS
THERAPY
SURGERY
Cushing’s
Nelson’s syndrome
bilateral adrenalectomy
tumor progression
Science & Technology
Life Sciences & Biomedicine
Endocrinology & Metabolism
Nelson's syndrome
Cushing's
bilateral adrenalectomy
tumor progression
CUSHINGS-DISEASE
STEREOTACTIC RADIOSURGERY
BILATERAL ADRENALECTOMY
MANAGEMENT
TUMORS
EXPERIENCE
MORTALITY
ADENOMAS
THERAPY
SURGERY
Endocrinology & Metabolism
1103 Clinical Sciences
1114 Paediatrics and Reproductive Medicine
Publication Status: Published
Article Number: UNSP dgz200
Online Publication Date: 2019-11-18
Appears in Collections:Department of Metabolism, Digestion and Reproduction