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

Reincke M, Albani A, Assie G, Bancos I, Brue T, Buchfelder M, Chabre O, Ceccato F, Daniele A, Detomas M, Di Dalmazi G, Elenkova A, Findling J, Grossman AB, Gomez-Sanchez CE, Heaney AP, Honegger J, Karavitaki N, Lacroix A, Laws ER, Losa M, Murakami M, Newell-Price J, Giraldi FP, Perez-Rivas LG, Pivonello R, Rainey WE, Sbiera S, Schopohl J, Stratakis CA, Theodoropoulou M, Van Rossum EFC, Valassi E, Zacharieva S, Rubinstein G, Ritzel K (2021)

Publication Type: Journal article, Review article

Publication year: 2021

Journal

Book Volume: 184

Pages Range: P1-P16

Journal Issue: 3

DOI: 10.1530/EJE-20-1088

Abstract

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, 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. Results: 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). 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.

Authors with CRIS profile

Involved external institutions

University of Bologna / Università di Bologna Italy (IT) University of Padua / Universita degli Studi di Padova Italy (IT) Medical College of Wisconsin (MCW) United States (USA) (US) Queen Mary University of London United Kingdom (GB) Julius-Maximilians-Universität Würzburg Germany (DE) University of Mississippi (Ole Miss) United States (USA) (US) Klinikum der Universität München (Großhadern und Innenstadt) Germany (DE) University of California Los Angeles (UCLA) United States (USA) (US) Eberhard Karls Universität Tübingen Germany (DE) Harvard University United States (USA) (US) Università Vita-Salute San Raffaele (UniSR) Italy (IT) Università degli studi di Milano Italy (IT) University of Birmingham United Kingdom (GB) Centre hospitalier de l'Université de Montréal (CHUM) Canada (CA) University of Sheffield United Kingdom (GB) University of Michigan United States (USA) (US) Eunice Kennedy Shriver National Institute of Child Health and Human Development United States (USA) (US) Erasmus University Medical Center (MC) Netherlands (NL) Autonomous University of Barcelona (UAB) / Universitat Autònoma de Barcelona Spain (ES) Aix-Marseille University / Aix-Marseille Université France (FR) Centre hospitalier universitaire de Grenoble France (FR) Università degli Studi di Napoli Federico II Italy (IT) Medical University Sofia / Медицински университет Bulgaria (BG) University of Paris 4 - Paris-Sorbonne / Université paris IV Paris-Sorbonne France (FR) Mayo Clinic United States (USA) (US)

How to cite

APA:

Reincke, M., Albani, A., Assie, G., Bancos, I., Brue, T., Buchfelder, M.,... Ritzel, K. (2021). Corticotroph tumor progression after bilateral adrenalectomy (Nelson's syndrome): systematic review and expert consensus recommendations. European Journal of Endocrinology, 184(3), P1-P16. https://doi.org/10.1530/EJE-20-1088

MLA:

Reincke, Martin, et al. "Corticotroph tumor progression after bilateral adrenalectomy (Nelson's syndrome): systematic review and expert consensus recommendations." European Journal of Endocrinology 184.3 (2021): P1-P16.

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