Prevalence of Nelson’s syndrome after bilateral adrenalectomy in patients with cushing’s disease: a systematic review and meta-analysis

Papakokkinou, Eleni; Piasecka, Marta; Carlsen, Hanne Krage; Chantzichristos, Dimitrios; Olsson, Daniel S; Dahlqvist, Per; Petersson, Maria; Berinder, Katarina; Bensing, Sophie; Höybye, Charlotte; Engström, Britt Edén; Burman, Pia; Follin, Cecilia; Petranek, David; Erfurth, Eva Marie; Wahlberg, Jeanette; Ekman, Bertil; Åkerman, Anna Karin; Schwarcz, Erik; Johannsson, Gudmundur; Falhammar, Henrik; Ragnarsson, Oskar

doi:10.1007/s11102-021-01158-z

Cited by 11 publications

(2 citation statements)

References 59 publications

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“…could remove theses lesions, and pituitary radiotherapy could control tumor progression in a majority of cases with unresectable growing remnants. In a recent meta-analysis, around 30% of patients exhibit tumor enlargement during follow-up, with only 20% developing a progressive tumor requiring intervention, either through pituitary surgery or radiotherapy [20].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

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

Bessiène,

Villa,

Bertagna

et al. 2024

Exp Clin Endocrinol Diabetes

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Since the first description 60 years ago of Nelson syndrome, 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 (CTPS) speeds can be obsersed. Using a simple metrics in patients with CTP, CTPS was reported to vary from few millimeters per year to up to 40 mm per year. Rapid CTPS/Nelson’s syndrome was associated with more severe Cushing, higher 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 to intermediate morning ACTH measurements probably provide a reliable way to detect early and manage fast growing tumors, and therefore to limit the complications.

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Section: Accepted Manuscriptmentioning

confidence: 99%

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

Bessiène,

Villa,

Bertagna

et al. 2024

Exp Clin Endocrinol Diabetes

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“…The prevalence of NS varies significantly between studies, influenced by the criteria used for its diagnosis, the length of follow-up and possible referral bias of the reporting centres. In a recent systematic review of 36 studies including 1316 CD patients treated with BLA, a mean NS prevalence of 26% at a median follow-up of 7 years was found [16].…”

Section: Introductionmentioning

confidence: 99%

Management of Nelson’s Syndrome

Fountas

Karavitaki

2022

Medicina

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Nelson’s syndrome is a potentially severe condition that may develop in patients with Cushing’s disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alone needs to be considered in cases requiring intervention and are poor surgical candidates. Observation is an option for patients with small lesions, not causing mass effects to vital adjacent structures but close follow-up is required for a timely detection of corticotroph tumour progression and for further treatment if required. To date, no medical therapy has been consistently proven to be effective in Nelson’s syndrome. Pharmacotherapy, however, should be considered when other management approaches have failed. A subset of patients with Nelson’s syndrome may develop further tumour growth after primary treatment, and, in some cases, a truly aggressive tumour behaviour can be demonstrated. In the absence of evidence-based guidance, the management of these cases is individualized and tailored to previously offered treatments. Temozolomide has been used in patients with aggressive Nelson’s with no consistent results. Development of tumour-targeted therapeutic agents are an unmet need for the management of aggressive cases of Nelson’s syndrome.

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Endocrine Hypertension

White

2023

Pediatric Hypertension

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Prevalence of Nelson’s syndrome after bilateral adrenalectomy in patients with cushing’s disease: a systematic review and meta-analysis

Cited by 11 publications

References 59 publications

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

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

Management of Nelson’s Syndrome

Endocrine Hypertension

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