ECE2019 Oral Communications Cushing's and acromegaly (5 abstracts)
Outcomes after primary treatment for Nelson’s syndrome: a study from 13 UK centres
Athanasios Fountas 1, , Eugenie Lim 4 , William M Drake 4 , Andrew Polson 5 , Mark Gurnell 5 , Niamh M Martin 6 , Khyatisha Seejore 7 , Robert D Murray 7 , James MacFarlane 8 , Rupa Ahluwalia 8 , Francesca Swords 8 , Muhammad Ashraf 9 , Aparna Pal 9 , Zhuomin Chong 10 , Marie Freel 10 , Tala Balafshan 11 , Tejpal S Purewal 11 , Rowena G Speak 12 , John Newell-Price 12 , Claire Higham 13 , Ziad Hussein 14 , Stephanie E Baldeweg 14 , Jolyon Dales 15 , Narendra Reddy 15 , Miles Levy 15 & Niki Karavitaki 1,
1Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK; 2Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK; 3University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK; 4Barts Health NHS Trust, London, UK; 5Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 6Imperial College Healthcare NHS Trust, London, UK; 7Leeds Teaching Hospital NHS Trust, Leeds, UK; 8Norfolk and Norwich University Hospitals NHS Foundation Trust, Norfolk, UK; 9Oxford University Hospitals NHS Foundation Trust, Oxford, UK; 10Queen Elizabeth University Hospital Glasgow, Glasgow, UK; 11Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK; 12Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK; 13The Christie NHS Foundation Trust, Manchester, UK; 14University College London Hospitals NHS Foundation Trust, London, UK; 15University Hospitals of Leicester NHS Trust, Leicester, UK.
Nelson’s syndrome (NS) is a potentially severe complication following bilateral adrenalectomy for Cushing’s disease (CD). Series assessing outcomes of treatments for NS are limited by small sample size, often short follow-up and variability of success criteria. We performed a UK multi-centre study aiming to review outcomes of primary treatment for NS. Clinical, laboratory, imaging data were collected. Kaplan-Meier method, log-rank test, Cox regression analysis were used for statistical analyses. 68 patients from 13 centres were included [58 females, median age at CD diagnosis 30 years (11-69)]. Management of CD included surgery+adrenalectomy (n=32, 8 had two and 1 had three operations), surgery+radiotherapy+adrenalectomy (n=15, 2 received >1 course of irradiation), radiotherapy +adrenalectomy (n=1), adrenalectomy (n=20). NS diagnosis relied on i) imaging (adenoma growth or identification of tumour if previous scan negative) combined or not with increasing ACTH levels and/or pigmentation (n=53), ii) only increasing ACTH levels and/or pigmentation (n=14) (no information for one patient). Diagnosis of NS was between 1969–2018 (in 59/68, after 1990) at median interval 3 years after adrenalectomy (3 months-32 years). Primary management of NS included surgery (n=10), radiotherapy (n=22), surgery+radiotherapy (n=18, one had also carmustine implant), observation (n=16), pasireotide (n=2). Imaging follow-up data were available for 64 patients; there was no significant difference in probability of tumour progression between the primary management groups (10-years cumulative probability of progression-free survival: total group 76%, surgery 75%, radiotherapy 83%, surgery+radiotherapy 73%, observation 68%). Based on cases with relevant available data, sex, age at CD diagnosis, adenoma size at diagnosis (micro/macro), presence of adenoma on imaging before adrenalectomy, extent of adrenalectomy (complete/or not), interval between adrenalectomy and NS diagnosis (<3 or ≥3 years) and diagnostic criteria for NS (positive imaging/or not) were not predictors of tumour progression. However, type of management of CD was predictor (P<0.05); thus, in comparison with surgery+adrenalectomy, hazard ratios for surgery+radiotherapy+adrenalectomy were 5.5 (95%CI 1.8–17.1) and for adrenalectomy 0.3 (95%CI 0.7–1.1). This finding persisted even after adjusting for type of primary treatment of NS. Median follow-up between NS diagnosis and last review was 16 years (0–48); 13 patients had died and in three the cause was related with NS. At 10-years follow-up, 24% of the Nelson’s tumours will show progress after various management approaches. Complexity of CD treatment, possibly reflecting corticotroph adenoma aggressiveness, is a significant predictor of Nelson’s tumour progress.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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