Corticotroph tumor progression after bilateral adrenalectomy: data from ERCUSYN

in Endocrine-Related Cancer
Authors:
Elena Valassi IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain
Universitat Internacional de Catalunya (UIC), Barcelona, Spain

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https://orcid.org/0000-0002-3864-0105
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Frédéric Castinetti Department of Endocrinology, Aix Marseille University, AP-HM, INSERM, Marseille Medical Genetics, Marmara Institute, La Conception Hospital, Marseille, France

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Amandine Ferriere Department of Endocrinology, Diabetes and Nutrition, University of Bordeaux, Bordeaux, France

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Stylianos Tsagarakis Department of Endocrinology, Diabetes and Metabolism, Evangelismos Hospital, Athens, Greece

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Richard A Feelders Erasmus Medical Center, Division of Endocrinology, Department of Internal Medicine, Rotterdam, The Netherlands

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Romana T Netea-Maier Division of Endocrinology, Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands

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Michael Droste Praxis für Endokrinologie Dr. med. Michael Droste, Oldenburg, Germany

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Christian J Strasburger Division of Clinical Endocrinology, Department of Medicine CCM, Charité-Universitätsmedizin, Berlin, Germany

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Dominique Maiter Service d’Endocrinologie et Nutrition, Cliniques universitaires Saint Luc, Brussels, Belgium

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Darko Kastelan Department of Endocrinology, University Hospital Zagreb, Zagreb, Croatia

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Philippe Chanson Institut National de la Santé et de la Recherche Médicale, U1185, Le Kremlin, Bicêtre, Paris, France

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Susan M Webb IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain
Universitat Autònoma de Barcelona (UAB), Barcelona, Spain

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Frank Demtröder Zentrum für Endokrinologie, Diabetologie, Rheumatologie Dr. Demtröder & Kollegen, Dortmund, Germany

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Valdis Pirags Paula Stradiņa klīniskā universitātes slimnīca, Riga, Latvia

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Olivier Chabre Hospitalier Universitaire, Grenoble, France

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Holger Franz Lohmann & Birkner Health Care Consultimg GmbH, Berlin, Germany

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Alicia Santos IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain
Universitat Autònoma de Barcelona (UAB), Barcelona, Spain

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Martin Reincke Medizinische Klinik UND Poliklinik IV, Campus Innestadt, Klinikum der Universität München, Ludwig-Maximilians-Universität München, Munich, Germany

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Correspondence should be addressed to E Valassi: EValassi@santpau.cat
DOI:
https://doi.org/10.1530/ERC-22-0074
Volume/Issue:
Volume 29: Issue 12
Article Type:
Research Article
Page Range:
681–691
Online Publication Date:
02 Nov 2022
Copyright:
© the author(s) 2022
Restricted access
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Corticotroph tumor progression after bilateral adrenalectomy/Nelson’s syndrome (CTP-BADX/NS) is a severe complication of bilateral adrenalectomy (BADX). The aim of our study was to investigate the prevalence, presentation and outcome of CTP-BADX/NS in patients with Cushing’s disease (CD) included in the European Registry on Cushing’s Syndrome (ERCUSYN). We examined data on 1045 CD patients and identified 85 (8%) who underwent BADX. Of these, 73 (86%) had follow-up data available. The median duration of follow-up since BADX to the last visit/death was 7 years (IQR 2–9 years). Thirty-three patients (45%) experienced CTP-BADX/NS after 3 years (1.5–6) since BADX. Cumulative progression-free survival was 73% at 3 years, 66% at 5 years and 46% at 10 years. CTP-BADX/NS patients more frequently had a visible tumor at diagnosis of CD than patients without CTP-BADX/NS (P < 0.05). Twenty-seven CTP-BADX/NS patients underwent surgery, 48% radiotherapy and 27% received medical therapy. The median time since diagnosis of CTP-BADX/NS to the last follow-up visit was 2 years (IQR, 1–5). Control of tumor progression was not achieved in 16 of 33 (48%) patients, of whom 8 (50%) died after a mean of 4 years. Maximum adenoma size at diagnosis of CD was associated with further tumor growth in CTP-BADX/NS despite treatment (P = 0.033). Diagnosis of CTP-BADX/NS, older age, greater UFC levels at diagnosis of CD and initial treatment predicted mortality. In conclusion, CTP-BADX/NS was reported in 45% of the ERCUSYN patients who underwent BADX, and control of tumor growth was reached in half of them. Future studies are needed to establish effective strategies for prevention and treatment.

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Print ISSN:
1351-0088
Online ISSN:
1479-6821
Copyright:
© the author(s) 2022
Page(s):
11
Received Date:
01 Sep 2022
Accepted Date:
23 Sep 2022
Print Publication Date:
01 Dec 2022
Online Publication Date:
02 Nov 2022
Keywords:
Cushing’s disease; Nelson’s syndrome; bilateral adrenalectomy; corticotroph adenoma

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