Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations - Archive ouverte HAL Access content directly
Journal Articles European Journal of Endocrinology Year : 2021

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

1 LMU - Ludwig-Maximilians-Universität München
2 IC UM3 (UMR 8104 / U1016) - Institut Cochin
3 Hôpital Cochin [AP-HP]
4 Mayo Clinic [Rochester]
5 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
6 MarMaRa - Institut Marseille Maladies Rares
7 Service d'endocrinologie, diabète, maladies métaboliques [Hôpital de la Conception - APHM]
8 Universitätsklinikum Erlangen [Erlangen]
9 CHU - Centre Hospitalier Universitaire [Grenoble]
10 Unipd - Università degli Studi di Padova = University of Padua
11 University of Würzburg = Universität Würzburg
12 University of Bologna/Università di Bologna
13 Medical University of Sofia [Bulgarie]
14 MCW - Medical College of Wisconsin [Milwaukee]
15 QMUL - Queen Mary University of London
16 UMMC - University of Mississippi Medical Center
17 School of Medicine [Los Angeles]
18 University of Tübingen
19 University of Birmingham [Birmingham]
20 OCDEM - Oxford Centre for Diabetes, Endocrinology and Metabolism
21 University Hospitals Birmingham [Birmingham, Royaume-Uni]
22 CHUM - Centre Hospitalier de l'Université de Montréal
23 HMS - Harvard Medical School [Boston]
24 IRCCS San Raffaele Scientific Institute [Milan, Italie]
25 TMDU - Tokyo Medical and Dental University [Japan]
26 University of Sheffield [Sheffield]
27 UNIMI - Università degli Studi di Milano = University of Milan
28 University of Naples Federico II = Università degli studi di Napoli Federico II
29 University of Michigan [Ann Arbor]
30 NICHD - Eunice Kennedy Shriver National Institute of Child Health and Human Development
31 Erasmus MC - Erasmus University Medical Center [Rotterdam]
32 Hospital de la Santa Creu i Sant Pau
Irina Bancos


Background Corticotroph tumor progression (CTP) leading to Nelson’s syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing’s disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing. Methods A systematic literature search was performed focusing on clinical studies and case series (≥5 patients). Definition, cumulative incidence, treatment and long-term outcomes of CTP/NS after BADX were analyzed using descriptive statistics. The results were presented and discussed at an interdisciplinary consensus workshop attended by international pituitary experts in Munich on October 28, 2018. Results Data covered definition and cumulative incidence (34 studies, 1275 patients), surgical outcome (12 studies, 187 patients), outcome of radiation therapy (21 studies, 273 patients), and medical therapy (15 studies, 72 patients). Conclusions We endorse the definition of CTP-BADX/NS as radiological progression or new detection of a pituitary tumor on thin-section MRI. We recommend surveillance by MRI after 3 months and every 12 months for the first 3 years after BADX. Subsequently, we suggest clinical evaluation every 12 months and MRI at increasing intervals every 2–4 years (depending on ACTH and clinical parameters). We recommend pituitary surgery as first-line therapy in patients with CTP-BADX/NS. Surgery should be performed before extrasellar expansion of the tumor to obtain complete and long-term remission. Conventional radiotherapy or stereotactic radiosurgery should be utilized as second-line treatment for remnant tumor tissue showing extrasellar extension

Dates and versions

hal-03662879 , version 1 (09-05-2022)



Martin Reincke, Adriana Albani, Guillaume Assie, Irina Bancos, Thierry Brue, et al.. Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations. European Journal of Endocrinology, 2021, 184 (3), pp.P1-P16. ⟨10.1530/EJE-20-1088⟩. ⟨hal-03662879⟩
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