From Nelson’s Syndrome to Corticotroph Tumor Progression Speed: An
                    Update

Laura Bessiène; Chiara Villa; Xavier Bertagna; Bertrand Baussart; Guillaume Assié

doi:10.1055/a-2359-8649

Experimental and Clinical Endocrinology & Diabetes, Inhaltsverzeichnis

Exp Clin Endocrinol Diabetes 2024; 132(10): 581-590
DOI: 10.1055/a-2359-8649

Review

From Nelson’s Syndrome to Corticotroph Tumor Progression Speed: An Update

Laura Bessiène

¹Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France

,

Chiara Villa

²Université Paris Cité, Institut Cochin, Inserm, CNRS, F-75014 Paris, France

³Department of Pathological Cytology and Anatomy, AP-HP, Hôpital Pitié-Salpétrière, F-75013 Paris, France

,

Xavier Bertagna

¹Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France

,

Bertrand Baussart

⁴Department of Neurosurgery, Pitié Salpétrière, AP-HP, Hôpital Pitié-Salpétrière, F-75013 Paris, France

,

Guillaume Assié

¹Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France

²Université Paris Cité, Institut Cochin, Inserm, CNRS, F-75014 Paris, France

› Institutsangaben

Abstract

Since the first description of Nelson syndrome 60 years ago, the way to consider corticotroph pituitary neuroendocrine tumors (PitNETs) after bilateral adrenalectomy has evolved. Today, it is globally acknowledged that only a subset of corticotroph PitNETs is aggressive.

After adrenalectomy, corticotroph tumor progression (CTP) occurs in about 30 to 40% of patients during a median follow-up of 10 years. When CTP occurs, various CTP speeds (CTPS) can be observed. Using simple metrics in patients with CTP, CTPS was reported to vary from a few millimeters to up to 40 mm per year. Rapid CTPS/ Nelson’s syndrome was associated with more severe Cushing’s disease, higher adrenocorticotropic hormone (ACTH) in the year following adrenalectomy, and higher Ki67 on pituitary pathology. Complications such as apoplexy, cavernous syndrome, and visual defects were associated with higher CTPS. During follow-up, early morning ACTH, absolute variations properly reflected CTPS. Finally, CTPS was not higher after than before adrenalectomy, suggesting that cortisol deprivation after adrenalectomy does not impact CTPS in a majority of patients.

Taken together, rapid CTPS/ Nelson’s syndrome probably reflects the intrinsic aggressiveness of some corticotroph PitNETs. The precise molecular mechanisms related to corticotroph PitNET aggressiveness remain to be deciphered. Regular MRIs combined with intermediate morning ACTH measurements probably provide a reliable way to detect early and manage fast-growing tumors and, therefore, limit the complications.

Keywords

PitNET - Nelson - Cushing - adrenalectomy

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Referenzen

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