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Start Over Descriptor "Nelson's syndrome" Publisher bioscientifica
9 results on '"Nelson's syndrome"'

1. Corticotroph tumor progression after bilateral adrenalectomy: data from ERCUSYN.

Author

Valassi, Elena, Castinetti, Frédéric, Ferriere, Amandine, Tsagarakis, Stylianos, Feelders, Richard A., Netea-Maier, Romana T., Droste, Michael, Strasburger, Christian J., Maiter, Dominique, Kastelan, Darko, Chanson, Philippe, Webb, Susan M., Demtröder, Frank, Pirags, Valdis, Chabre, Olivier, Franz, Holger, Santos, Alicia, and Reincke, Martin

Subjects
*CANCER invasiveness, *CUSHING'S syndrome, *ADRENALECTOMY, *TUMOR growth, *TUMOR diagnosis
Abstract

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 (IQ R 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 who m 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 re ached in half of them. Future studies are needed to establish effective strategies for prevention and treatment. [ABSTRACT FROM AUTHOR]

Published
2022
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2. Corticotroph tumor progression after bilateral adrenalectomy: data from ERCUSYN.

Author

UCL - SSS/IREC/EDIN - Pôle d'endocrinologie, diabète et nutrition, UCL - (SLuc) Service d'endocrinologie et de nutrition, Valassi, Elena, Castinetti, Frédéric, Ferriere, Amandine, Tsagarakis, Stylianos, Feelders, Richard A, Netea-Maier, Romana T, Droste, Michael, Strasburger, Christian J, Maiter, Dominique, Kastelan, Darko, Chanson, Philippe, Webb, Susan M, Demtröder, Frank, Pirags, Valdis, Chabre, Olivier, Franz, Holger, Santos, Alicia, Reincke, Martin, UCL - SSS/IREC/EDIN - Pôle d'endocrinologie, diabète et nutrition, UCL - (SLuc) Service d'endocrinologie et de nutrition, Valassi, Elena, Castinetti, Frédéric, Ferriere, Amandine, Tsagarakis, Stylianos, Feelders, Richard A, Netea-Maier, Romana T, Droste, Michael, Strasburger, Christian J, Maiter, Dominique, Kastelan, Darko, Chanson, Philippe, Webb, Susan M, Demtröder, Frank, Pirags, Valdis, Chabre, Olivier, Franz, Holger, Santos, Alicia, and Reincke, Martin

Abstract

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.

Published
2022

3. Corticotroph tumor progression after bilateral adrenalectomy: data from ERCUSYN

Author

Elena Valassi, Frédéric Castinetti, Amandine Ferriere, Stylianos Tsagarakis, Richard A Feelders, Romana T Netea-Maier, Michael Droste, Christian J Strasburger, Dominique Maiter, Darko Kastelan, Philippe Chanson, Susan M Webb, Frank Demtröder, Valdis Pirags, Olivier Chabre, Holger Franz, Alicia Santos, Martin Reincke, UCL - SSS/IREC/EDIN - Pôle d'endocrinologie, diabète et nutrition, UCL - (SLuc) Service d'endocrinologie et de nutrition, Internal Medicine, Universitat Internacional de Catalunya [Barcelona] (UIC), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Marseille Maladies Rares (MarMaRa), Aix Marseille Université (AMU), Service d'endocrinologie, diabète, maladies métaboliques [Hôpital de la Conception - APHM], Université de Bordeaux (UB), Evangelismos Athens General Hospital, Erasmus University Medical Center [Rotterdam] (Erasmus MC), Radboud University Medical Center [Nijmegen], Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Cliniques Universitaires Saint-Luc [Bruxelles], University Hospital Centre Zagreb, Partenaires INRAE, Institut National de la Santé et de la Recherche Médicale (INSERM), Universitat Autònoma de Barcelona (UAB), CHU Grenoble, Ludwig-Maximilians-Universität München (LMU), and ERCUSYN was set up with funding from the EU (PHP 800200) and beensupported by unrestricted grants from Novartis, Ipsen, HRA and theEuropean Society of Endocrinology.

Subjects
Cancer Research, Cushing's diseaseNelson's syndrome, bilateral adrenalectomy, corticotroph adenoma, Nelson’s syndrome, Endocrinology, Diabetes and Metabolism, Adrenalectomy, Rare cancers Radboud Institute for Molecular Life Sciences [Radboudumc 9], [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, Cushing’s disease, Nelson Syndrome, Endocrinology, SDG 3 - Good Health and Well-being, Oncology, Humans, Pituitary ACTH Hypersecretion, Corticotrophs, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

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 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.

Published
2022
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5. Outcomes after primary treatment for Nelson's syndrome: a study from 13 UK centres

Author

Eugenie Lim, Ziad Hussein, Tejpal Purewal, Francesca Swords, Robert D Murray, Miles Levy, Stephanie E Baldeweg, Niki Karavitaki, Athanasios Fountas, Marie Freel, Rupa Ahluwalia, Andrew Polson, Niamh M. Martin, Khyatisha Seejore, Rowena Speak, Jolyon Dales, Tala Balafshan, Narendra Reddy, Muhammad Ashraf, Mark Gurnell, James MacFarlane, Zhuomin Chong, William Drake, John Newell-Price, Aparna Pal, and Claire E Higham

Subjects
medicine.medical_specialty, business.industry, Family medicine, Nelson's syndrome, medicine, Primary treatment, medicine.disease, business
Published
2019
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6. Somatic mutations in USP8 are frequent events in pituitary tumors causing Nelson's syndrome

Author

Benno Küsters, Timo Deutschbein, Günter K. Stalla, Troy H Puar, Francesco Ferraù, Marily Theodoropoulou, Maria Candida Barisson Vilares Fragoso, Monica R Gadelha, Felix Beuschlein, Guillaume Assié, Jérôme Bertherat, Julia Fazel, Márta Korbonits, Martin Reincke, Luis G. Perez-Rivas, Mareike R. Stieg, and Ad Hermus

Subjects
Somatic cell, business.industry, Nelson's syndrome, Pituitary tumors, medicine, Cancer research, medicine.disease, business
Published
2017
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