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

Research output: Contribution to journalReview articlepeer-review

Authors

  • Martin Reincke
  • Adriana Albani
  • Guillaume Assie
  • Irina Bancos
  • Thierry Brue
  • Michael Buchfelder
  • Olivier Chabre
  • Filippo Ceccato
  • Andrea Daniele
  • Mario Detomas
  • Guido Di Dalmazi
  • Atanaska Elenkova
  • James Findling
  • Ashley Grossman
  • Celso Gomez-Sanchez
  • Anthony Heaney
  • Jurgen Honegger
  • André Lacroix
  • Edward Laws
  • Marco Losa
  • Masanori Murakami
  • John Newell-Price
  • Francesca Pecori
  • Luis G. Perez-Rivas
  • Rosario Pivonello
  • William Rainey
  • Silviu Sbiera
  • Jochen Schopohl
  • Constantine A Stratakis
  • Marily Theodoropoulou
  • Elisabeth Van Rossum
  • Elena Valassi
  • Sabina Zacharieva
  • German Rubinstein
  • Katrin Ritzel

Colleges, School and Institutes

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

Details

Original languageEnglish
Pages (from-to)P1–P16
Number of pages16
JournalEuropean Journal of Endocrinology
Volume184
Issue number3
Early online date31 Jan 2021
Publication statusPublished - Mar 2021