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

Martin Reincke 1 Adriana Albani 1 Guillaume Assie 2, 3 Irina Bancos 4 Thierry Brue 5, 6, 7 Michael Buchfelder 8 Olivier Chabre 9 Filippo Ceccato 10 Andrea Daniele 10 Mario Detomas 11 Guido Di Dalmazi 12 Atanaska Elenkova 13 James Findling 14 Ashley Grossman 15 Celso Gomez-Sanchez 16 Anthony Heaney 17 Juergen Honegger 18 Niki Karavitaki 19, 20, 21 Andre Lacroix 22 Edward Laws 23 Marco Losa 24 Masanori Murakami 1, 25 John Newell-Price 26 Francesca Pecori Giraldi 27 Luis Pérez‐rivas 1 Rosario Pivonello 28 William Rainey 29 Silviu Sbiera 11 Jochen Schopohl 1 Constantine Stratakis 30 Marily Theodoropoulou 1 Elisabeth van Rossum 31 Elena Valassi 32 Sabina Zacharieva 13 German Rubinstein 1 Katrin Ritzel 1 
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
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Submitted on : Monday, May 9, 2022 - 4:15:57 PM
Last modification on : Wednesday, November 30, 2022 - 4:14:07 PM

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Martin Reincke, Adriana Albani, Guillaume Assie, Irina Bancos, Thierry Brue, et al.. Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations. European Journal of Endocrinology, 2021, 184 (3), pp.P1-P16. ⟨10.1530/EJE-20-1088⟩. ⟨hal-03662879⟩



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