{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:46:06Z","timestamp":1790696766067,"version":"4.1.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"3","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,1,12]]},"abstract":"<jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Corticotroph tumor progression (CTP) leading to Nelson\u2019s syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing\u2019s disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>A systematic literature search was performed focusing on clinical studies and case series (\u22655 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.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>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).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>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\u20134 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<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-20-1088","type":"journal-article","created":{"date-parts":[[2021,1,12]],"date-time":"2021-01-12T23:05:27Z","timestamp":1610492727000},"page":"P1-P16","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":49,"title":["Corticotroph tumor progression after bilateral adrenalectomy (Nelson\u2019s syndrome): systematic review and expert consensus recommendations"],"prefix":"10.1093","volume":"184","author":[{"given":"Martin","family":"Reincke","sequence":"first","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana","family":"Albani","sequence":"additional","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Assie","sequence":"additional","affiliation":[{"name":"2Department of Endocrinology, Universit\u00e9 de Paris, Institut Cochin, INSERM, CNRS, Center for Rare Adrenal Diseases, H\u00f4pital Cochin, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irina","family":"Bancos","sequence":"additional","affiliation":[{"name":"3Division of Endocrinology, Mayo Clinic Minnesota, Diabetes, Metabolism, Nutrition, Rochester, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thierry","family":"Brue","sequence":"additional","affiliation":[{"name":"4Aix-Marseille Universit\u00e9, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), U1251, Marseille Medical Genetics (MMG), Institut MarMaRa and Endocrinology Department, Conception Hospital, Assistance Publique-H\u00f4pitaux de Marseille (APHM), Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Buchfelder","sequence":"additional","affiliation":[{"name":"5Universit\u00e4tsklinikum Erlangen, Neurochirurgische Klinik, Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Chabre","sequence":"additional","affiliation":[{"name":"6CHU Grenoble-Alpes, Unit of Endocrinology, Pavillon des Ecrins, Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1456-8716","authenticated-orcid":true,"given":"Filippo","family":"Ceccato","sequence":"additional","affiliation":[{"name":"7Department of Medicine, University of Padova, Padova, Veneto, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Daniele","sequence":"additional","affiliation":[{"name":"7Department of Medicine, University of Padova, Padova, Veneto, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Detomas","sequence":"additional","affiliation":[{"name":"8Division of Endocrinology and Diabetology, Department of Internal Medicine, University of W\u00fcrzburg, Wurzburg, Bayern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guido","family":"Di Dalmazi","sequence":"additional","affiliation":[{"name":"9Department of Medical and Surgical Sciences, Endocrinology and Diabetes Prevention and Care Unit, University of Bologna, S. Orsola Policlinic, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atanaska","family":"Elenkova","sequence":"additional","affiliation":[{"name":"10Department of Endocrinology, Medical University Sofia, Sofia, Bulgaria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Findling","sequence":"additional","affiliation":[{"name":"11Division of Endocrinology and Molecular Medicine, Medical College of Wisconsin, Menomonee Falls, Wisconsin, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley B","family":"Grossman","sequence":"additional","affiliation":[{"name":"12Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Centre for Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celso E","family":"Gomez-Sanchez","sequence":"additional","affiliation":[{"name":"13Department of Pharmacology and Toxicology and Medicine, Endocrine Service, G.V. Montgomery VA Medical Center, University of Mississippi Medical Center, Jackson, Mississippi, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony P","family":"Heaney","sequence":"additional","affiliation":[{"name":"14Division of Endocrinology, Medical Director, Pituitary & Neuroendocrine Tumor Program, UCLA School of Medicine, Los Angeles, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juergen","family":"Honegger","sequence":"additional","affiliation":[{"name":"15Department of Neurosurgery, University of T\u00fcbingen, T\u00fcbingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4696-0643","authenticated-orcid":true,"given":"Niki","family":"Karavitaki","sequence":"additional","affiliation":[{"name":"16Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham"},{"name":"17Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners"},{"name":"18Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4137-3025","authenticated-orcid":true,"given":"Andre","family":"Lacroix","sequence":"additional","affiliation":[{"name":"19Division of Endocrinology, Department of Medicine, Centre hospitalier de l\u2019Universit\u00e9 de Montr\u00e9al (CHUM), Montr\u00e9al, Qu\u00e9bec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward R","family":"Laws","sequence":"additional","affiliation":[{"name":"20Pituitary\/Neuroendocrine Center, Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Losa","sequence":"additional","affiliation":[{"name":"21Department of Neurosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Murakami","sequence":"additional","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"},{"name":"22Department of Molecular Endocrinology and Metabolism, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Newell-Price","sequence":"additional","affiliation":[{"name":"23Dept of Oncology and Metabolism, The Medical School University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6065-7803","authenticated-orcid":true,"given":"Francesca","family":"Pecori Giraldi","sequence":"additional","affiliation":[{"name":"24Department of Clinical Sciences & Community Health, University of Milan Neuroendocrinology Research Laboratory, Instituto Auxologico Italiano IRCCS, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis G","family":"P\u00e9rez\u2010Rivas","sequence":"additional","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosario","family":"Pivonello","sequence":"additional","affiliation":[{"name":"25Dipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Universit\u00e0 Federico II di Napoli, Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William E","family":"Rainey","sequence":"additional","affiliation":[{"name":"26Departments of Molecular & Integrative Physiology and Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silviu","family":"Sbiera","sequence":"additional","affiliation":[{"name":"8Division of Endocrinology and Diabetology, Department of Internal Medicine, University of W\u00fcrzburg, Wurzburg, Bayern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Schopohl","sequence":"additional","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constantine A","family":"Stratakis","sequence":"additional","affiliation":[{"name":"27Section on Genetics & Endocrinology Eunice Kennedy Shriver National Insitute of Child Health & Human Development (NICHD) National Institute of Health (NIH), NIH Clinical Research Center, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marily","family":"Theodoropoulou","sequence":"additional","affiliation":[{"name":"1Klinikum der Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Medizinische Klinik und Poliklinik IV, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth F C","family":"van Rossum","sequence":"additional","affiliation":[{"name":"28Department of Internal Medicine, division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Valassi","sequence":"additional","affiliation":[{"name":"29IIB-Sant Pau and Department of Endocrinology\/Medicine, Hospital Sant Pau, UAB, and Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, 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