Corticotroph Tumor Progression after Bilateral Adrenalectomy (Nelson’s syndrome): Systematic Review and Expert Consensus Recommendations

in European Journal of Endocrinology
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  • 1 M Reincke, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 2 A Albani, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 3 G Assie, Department of Endocrinology, Center for Rare Adrenal Diseases, Hopital Cochin, Universite de Paris, Institut Cochin, INSERM, CNRS, F-75014 Paris, Paris, France
  • 4 I Bancos, Division of Endocrinology, Diabetes, Metabolism, Nutrition, Mayo Clinic Minnesota, Rochester, United States
  • 5 T Brue, Marseille Medical Genetics (MMG), Institut MarMaRa and Endocrinology Department, Conception Hospital, Assistance Publique-Hopitaux de Marseille (APHM), Aix-Marseille Universite, Institut National de la Sante et de la Recherche Medicinale (INSERM), U1251, Marseille, France
  • 6 M Buchfelder, Neurochirurgische Klinik, Universitätsklinikum Erlangen, Erlangen, Germany
  • 7 O Chabre, Unit of Endocrinology, Pavillon des Ecrins, CS 102017, CHU Grenoble-Alpes, Grenoble, France
  • 8 F Ceccato, Endocrinology Unit, Department of Medicine, University of Padova, Padova, Italy
  • 9 A Daniele, Endocrinology Unit, Department of Medicine, University of Padova, Padova, Italy
  • 10 M Detomas, Division of Endocrinology and Diabetology, Department of Internal Medicine, University of Würzburg, Wurzburg, Germany
  • 11 G Di Dalmazi, Department of Medical and Surgical Science, University of Bologna, Orsola-Malpighi Hospital, Endocrinology and Diabetes Prevention Care Unit, Bologna, Italy
  • 12 A Elenkova, Clinical Center of Endocrinology and Gerontology, Medical University Sofia, Sofia, 1431, Bulgaria
  • 13 J Findling, Medicine, Medical College of Wisconsi, Menomonee Falls, 53051, United States
  • 14 A Grossman, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Centre for Endocrinology, Queen Mary University of London Barts and The London School of Medicine and Dentistry, London, United Kingdom of Great Britain and Northern Ireland
  • 15 C Gomez-Sanchez, Endocrinology, University of Mississippi Medical Center, Jackson, 39216, United States
  • 16 A Heaney, Division of Endocrinology, UCLA, Los Angeles, United States
  • 17 J Honegger, Dept of Neurosurgery, Eberhard Karls University of Tubingen, Tuebingen, 72076, Germany
  • 18 N Karavitaki, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom of Great Britain and Northern Ireland
  • 19 A Lacroix, Division of Endocrinology, Department of Medicine, University of Montreal Hospital Centre, Montreal, Canada
  • 20 E Laws, Pituitary/Neuroendocrine Center, Brigham and Women’s Hospital, Harvard Medical School, Boston, United States
  • 21 M Losa, Neurosurgery, IRCCS San Raffaele, Milano, 20132, Italy
  • 22 M Murakami, Molecular Endocrinology and Metabolism, Tokyo Medical and Dental University, Tokyo, Japan
  • 23 J Newell-Price, Academic Unit of Diabetes, Endocrinology and Metabolism, University of Sheffield, Sheffield, United Kingdom of Great Britain and Northern Ireland
  • 24 F Pecori Giraldi, Department of Clinical Sciences and Community Health, Universita degli Studi di Milano, Milano, Italy
  • 25 L Pérez‐Rivas, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 26 R Pivonello, Department of Molecular and Clinical Endocrnology and Oncology, Federico II University, Naples, 80131, Italy
  • 27 W Rainey, Department of Molecular and Integrative Physiology and Department of Internal Medicine, University of Michigan, Ann Arbor, United States
  • 28 S Sbiera, Division of Endocrinology and Diabetology, Department of Internal Medicine, University of Würzburg, Wurzburg, Germany
  • 29 J Schopohl, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 30 C Stratakis, NICHD, NIH, Bethesda, Md 20892, United States
  • 31 M Theodoropoulou, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 32 E van Rossum, Department of Internal Medicine, division of Endocrinology, Erasmus MC, Rotterdam, Netherlands
  • 33 E Valassi, IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital de Sant Pau, Barcelona, Spain
  • 34 S Zacharieva, Clinical Center of Endocrinology and Gerontology, Medical University - Sofia, Sofia, Bulgaria
  • 35 G Rubinstein, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany
  • 36 K Ritzel, Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximillians-Universität München, Munich, Germany

Correspondence: Martin Reincke, Email: martin.reincke@med.uni-muenchen.de
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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, 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 28th, 2018.

RESULTS: Data covered definition and 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.

 

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