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

Autor:	Bessiène L; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France., Villa C; Université Paris Cité, Institut Cochin, Inserm, CNRS, F-75014 Paris, France.; Department of Pathological Cytology and Anatomy, AP-HP, Hôpital Pitié-Salpétrière, F-75013 Paris, France., Bertagna X; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France., Baussart B; Department of Neurosurgery, Pitié Salpétrière, AP-HP, Hôpital Pitié-Salpétrière, F-75013 Paris, France., Assié G; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, F-75014 Paris, France.; Université Paris Cité, Institut Cochin, Inserm, CNRS, F-75014 Paris, France.
Jazyk:	angličtina
Zdroj:	Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association [Exp Clin Endocrinol Diabetes] 2024 Oct; Vol. 132 (10), pp. 581-590. Date of Electronic Publication: 2024 Jul 03.
DOI:	10.1055/a-2359-8649
Abstrakt:	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. Competing Interests: We declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported. (Thieme. All rights reserved.)
Databáze:	MEDLINE
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