Evidence map›Paper›PMID 33444221›Full record

SynthesisEuropean journal of endocrinology2021

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

Martin Reincke, Adriana Albani, Guillaume Assie, Irina Bancos, Thierry Brue, Michael Buchfelder, Olivier Chabre, Filippo Ceccato, Andrea Daniele, Mario Detomas and 26 more

Open access · bronzeAbstract readSystematic ReviewConsensus Statement
In one paragraph

Synthesis in European journal of endocrinology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
29citing papers in PubMed, 3 pooled it
6.6field-weighted citation impact, top 2% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

29 citing papers in PubMed, 3 syntheses or guidelines pooled it, 55 citations in OpenAlex.

  1. Pooled it
  2. Guideline
  3. Pooled it
  4. Article
  5. Article
  6. Article
  7. Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025
    Review
  8. Article
  9. Article
  10. Cushing syndrome.Nature reviews. Disease primers · 2025
    Review
  11. Article
  12. Seliciclib: A New Treatment for Cushing's Disease?TouchREVIEWS in endocrinology · 2024
    Article
  13. Review
  14. Article
  15. Review
  16. Aggressive Pituitary Tumors and Pituitary Carcinomas: From Pathology to Treatment.The Journal of clinical endocrinology and metabolism · 2023
    Article
  17. Review
  18. Review
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

36 authors at 20 institutions in 10 countries.

Martin ReinckeKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Adriana AlbaniKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Guillaume AssieDepartment of Endocrinology, Université de Paris, Institut Cochin, INSERM, CNRS, Center for Rare Adrenal Diseases, Hôpital Cochin, Paris, France.
Irina BancosDivision of Endocrinology, Mayo Clinic Minnesota, Diabetes, Metabolism, Nutrition, Rochester, Minnesota, USA.
Thierry BrueAix-Marseille Université, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Institut MarMaRa and Endocrinology Department, Conception Hospital, Assistance Publique-Hôpitaux de Marseille (APHM), Marseille, France.
Michael BuchfelderUniversitätsklinikum Erlangen, Neurochirurgische Klinik, Erlangen, Germany.
Olivier ChabreCHU Grenoble-Alpes, Unit of Endocrinology, Pavillon des Ecrins, Grenoble, France.
Filippo CeccatoDepartment of Medicine, University of Padova, Padova, Veneto, Italy.ORCID 0000-0003-1456-8716
Andrea DanieleDepartment of Medicine, University of Padova, Padova, Veneto, Italy.
Mario DetomasDivision of Endocrinology and Diabetology, Department of Internal Medicine, University of Würzburg, Wurzburg, Bayern, Germany.
Guido Di DalmaziDepartment of Medical and Surgical Sciences, Endocrinology and Diabetes Prevention and Care Unit, University of Bologna, S. Orsola Policlinic, Bologna, Italy.
Atanaska ElenkovaDepartment of Endocrinology, Medical University Sofia, Sofia, Bulgaria.
James FindlingDivision of Endocrinology and Molecular Medicine, Medical College of Wisconsin, Menomonee Falls, Wisconsin, USA.
Ashley B GrossmanOxford 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.
Celso E Gomez-SanchezDepartment of Pharmacology and Toxicology and Medicine, Endocrine Service, G.V. Montgomery VA Medical Center, University of Mississippi Medical Center, Jackson, Mississippi, USA.
Anthony P HeaneyDivision of Endocrinology, Medical Director, Pituitary & Neuroendocrine Tumor Program, UCLA School of Medicine, Los Angeles, California, USA.
Juergen HoneggerDepartment of Neurosurgery, University of Tübingen, Tübingen, Germany.
Niki KaravitakiInstitute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham.ORCID 0000-0002-4696-0643
Andre LacroixDivision of Endocrinology, Department of Medicine, Centre hospitalier de l'Université de Montréal (CHUM), Montréal, Québec, Canada.ORCID 0000-0003-4137-3025
Edward R LawsPituitary/Neuroendocrine Center, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Marco LosaDepartment of Neurosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milano, Italy.
Masanori MurakamiKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
John Newell-PriceDept of Oncology and Metabolism, The Medical School University of Sheffield, Sheffield, UK.
Francesca Pecori GiraldiDepartment of Clinical Sciences & Community Health, University of Milan Neuroendocrinology Research Laboratory, Instituto Auxologico Italiano IRCCS, Milan, Italy.ORCID 0000-0001-6065-7803
Luis G Pérez-RivasKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Rosario PivonelloDipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Università Federico II di Napoli, Naples, Italy.
William E RaineyDepartments of Molecular & Integrative Physiology and Medicine, University of Michigan, Ann Arbor, Michigan, USA.
Silviu SbieraDivision of Endocrinology and Diabetology, Department of Internal Medicine, University of Würzburg, Wurzburg, Bayern, Germany.
Jochen SchopohlKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Constantine A StratakisSection 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.
Marily TheodoropoulouKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Elisabeth F C van RossumDepartment of Internal Medicine, division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.
Elena ValassiIIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau, UAB, and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain.
Sabina ZacharievaDepartment of Endocrinology, Medical University Sofia, Sofia, Bulgaria.
German RubinsteinKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Katrin RitzelKlinikum der Ludwig-Maximilians-Universität München, Medizinische Klinik und Poliklinik IV, Munich, Germany.
Ludwig-Maximilians-Universität München · DEMedical University of Sofia · BGUniversity of Padua · ITUniversity of Würzburg · DEBrigham and Women's Hospital · USCentre Hospitalier de l’Université de Montréal · CACentre Hospitalier Universitaire de Grenoble · FRCentre National de la Recherche Scientifique · FRErasmus MC · NLEunice Kennedy Shriver National Institute of Child Health and Human Development · USInserm · FRInstituto de Salud Carlos III · ESMayo Clinic · USMedical College of Wisconsin · USQueen Mary University of London · GBTokyo Medical and Dental University · JPUniversitätsklinikum Erlangen · DEUniversity Hospitals Birmingham NHS Foundation Trust · GBUniversity of Bologna · ITUniversity of Michigan · US

Funding

Tracking and Evaluation CoreU54GM115428 · NIGMS · UNIVERSITY OF MISSISSIPPI MED CTR · PI GOMEZ-SANCHEZ, CELSO ENRIQUE · 2016 to 2025
$38.2M
Molecular Genetics of Endocrine Tumors and Related DisordersZIAHD008920 · NICHD · EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT · PI STRATAKIS, CONSTANTINE A. · 2012 to 2021
$25.8M
Adrenal cell ATP1A1 mutations and mechanisms of aldosterone biosynthesisR01HL144847 · NHLBI · UNIVERSITY OF MISSISSIPPI MED CTR · PI GOMEZ-SANCHEZ, CELSO ENRIQUE · 2019 to 2022
$1.3M
Regulation of Mineralocorticoid Receptor ActionI01BX004681 · VA · G V SONNY MONTGOMERY VA MEDCIAL CENTER · PI GOMEZ-SANCHEZ, CELSO ENRIQUE · 2021 to 2023
–
BLRD VA I01 BX004681NHLBI NIH HHS R01 HL144847NIGMS NIH HHS U54 GM115428
6 · The paper itself

Abstract

backgroundCorticotroph 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.

methodsA 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.

resultsData 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).

conclusionsWe 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.

Indexed as

ACTH-Secreting Pituitary AdenomaAdenomaAdrenalectomyDisease ProgressionHumansNelson Syndrome

Identifiers

PMID33444221
PMCPMC8060870
OpenAlexW3118275010

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.