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Background <p>Nelson syndrome (NS), or corticotroph tumor progression after bilateral adrenalectomy (CTP-BADX/NS), is a serious complication in patients with Cushing disease (CD) following BADX. Surgical tumor removal is the recommended treatment, though adjuvant therapies may be necessary.</p> Aim of the study <p>To evaluate clinical, radiological, and hormonal features of CD patients after BADX, identify risk factors for CTP-BADX/NS and assessed treatment outcome and cardio-metabolic complications.</p> Methods <p>Retrospective study of 30 patients (male/female: 9/21; median age at CD diagnosis: 33 years, IQR 27–42) who underwent BADX and had a minimum follow-up of 18 months. Data were collected at diagnosis and during follow-up (6, 24 months and last visit).</p> Results <p>Over a median follow-up of 135 months, 9/30 patients (30%) developed NS, median 60 months after BADX. NS patients had earlier CD diagnosis and higher ACTH levels two years post-BADX [458 ng/L (IQR 245–723) vs. 146 ng/L (61–247), <i>p</i> = 0.020]. They also took lower fludrocortisone [0.05&#xa0;mg/day vs. 0.1&#xa0;mg/day, <i>p</i> = 0.001] and tended to use less hydrocortisone [20&#xa0;mg/day [20–25] vs. 30 [25–30], <i>p</i> = 0.06]. Pre-BADX stereotactic radiosurgery (SRS) was more frequent in non-NS patients (52% vs. 22%, <i>p</i> = 0.11).</p> <p>Hypertension was more common in NS patients (78% vs 43%), but diabetes less so (33% vs 48%). In the CTP-BADX group, 6/9 required pituitary surgery and/or radiotherapy; medical therapy was used in 5 patients with varied results.</p> Conclusion <p>CTP-BADX/NS occurred in 30% of cases in our cohort. Higher ACTH post-BADX and younger age at CD onset may predict NS. No hormonal or radiological markers reliably predicted tumor progression. SRS before BADX and higher hydrocortisone doses might offer protection. Tumor control often needed a multimodal approach, with limited success from medical therapy alone.</p>

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Clinical presentation, predictive factors and management of patients with Nelson syndrome: a retrospective study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年9月26日

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  • Pierluigi Mazzeo,
  • Giulia Bovo,
  • Alessandro Mondin,
  • Giacomo Voltan,
  • Renzo Manara,
  • Mario Caccese,
  • Luca Denaro,
  • Filippo Ceccato,
  • Mattia Barbot

摘要

Background

Nelson syndrome (NS), or corticotroph tumor progression after bilateral adrenalectomy (CTP-BADX/NS), is a serious complication in patients with Cushing disease (CD) following BADX. Surgical tumor removal is the recommended treatment, though adjuvant therapies may be necessary.

Aim of the study

To evaluate clinical, radiological, and hormonal features of CD patients after BADX, identify risk factors for CTP-BADX/NS and assessed treatment outcome and cardio-metabolic complications.

Methods

Retrospective study of 30 patients (male/female: 9/21; median age at CD diagnosis: 33 years, IQR 27–42) who underwent BADX and had a minimum follow-up of 18 months. Data were collected at diagnosis and during follow-up (6, 24 months and last visit).

Results

Over a median follow-up of 135 months, 9/30 patients (30%) developed NS, median 60 months after BADX. NS patients had earlier CD diagnosis and higher ACTH levels two years post-BADX [458 ng/L (IQR 245–723) vs. 146 ng/L (61–247), p = 0.020]. They also took lower fludrocortisone [0.05 mg/day vs. 0.1 mg/day, p = 0.001] and tended to use less hydrocortisone [20 mg/day [20–25] vs. 30 [25–30], p = 0.06]. Pre-BADX stereotactic radiosurgery (SRS) was more frequent in non-NS patients (52% vs. 22%, p = 0.11).

Hypertension was more common in NS patients (78% vs 43%), but diabetes less so (33% vs 48%). In the CTP-BADX group, 6/9 required pituitary surgery and/or radiotherapy; medical therapy was used in 5 patients with varied results.

Conclusion

CTP-BADX/NS occurred in 30% of cases in our cohort. Higher ACTH post-BADX and younger age at CD onset may predict NS. No hormonal or radiological markers reliably predicted tumor progression. SRS before BADX and higher hydrocortisone doses might offer protection. Tumor control often needed a multimodal approach, with limited success from medical therapy alone.