Gamma knife radiosurgery in patients with Nelson’s syndrome

Abstract

Purpose

Nelson’s syndrome (NS) is a long-term complication of bilateral adrenalectomy in patients with Cushing’s disease. The best therapeutic strategy in NS has not been well defined. Gamma knife radiosurgery (GKRS) is very effective to stop the growth of the pituitary adenoma, which is the main goal of the treatment of patients with NS. We report the largest series of patients with NS treated by GKRS at a single center.

Methods

The study was an observational, retrospective analysis of 28 consecutive patients with NS treated by GKRS in our department between 1995 and 2019. All patients had a growing ACTH-secreting pituitary adenoma. The main outcome of the study was to assess by the Kaplan–Meier method the risk of tumor progression after GKRS.

Results

The median follow-up after GKRS treatment was 98 months (IQR 61–155 months, range 7–250 months). Two patients (7.1%) had a recurrence of disease during follow-up. The 10-year progression-free survival was 91.7% (95% CI 80.5–100%). No patient had deterioration of visual function or oculomotor function after GKRS. New onset of hypogonadism and hypothyroidism occurred in 18.8% and 14.3% of the patients at risk.

Conclusion

Our study confirms that GKRS may stop the tumor growth in the majority of patients with NS, even though very aggressive adenomas may ultimately escape this treatment. Safety of GKRS was good in our experience, but due attention must be paid to planning the distribution of radiation to critical structures, especially in patients previously treated by radiation.

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Funding

This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.

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Correspondence to M. Losa.

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The authors declare that they have no conflict of interest.

Research involving human participants

All procedures performed during this observational, retrospective study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Each patient gave informed consent to the treatment with gamma knife radiosurgery.

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The dataset generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Losa, M., Detomas, M., Bailo, M. et al. Gamma knife radiosurgery in patients with Nelson’s syndrome. J Endocrinol Invest (2021). https://doi.org/10.1007/s40618-021-01531-6

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Keywords

  • Pituitary neoplasm
  • Bilateral adrenalectomy
  • Cushing’s disease
  • Radiosurgery
  • Adrenocorticotropin