Gamma knife radiosurgery in patients with Nelson’s syndrome

• Published in: Journal of endocrinological investigation 2021-10, Vol.44 (10), p.2243-2251
• Main Authors: Losa, M., Detomas, M., Bailo, M., Barzaghi, L. R., Albano, L., Piloni, M., Pagnano, A., Pedone, E., Mortini, P.
• Format: Article
• Language: English
• Subjects: Adenoma; Adenoma - pathology; Adenoma - surgery; Adolescent; Adrenalectomy; Adrenocorticotropic hormone; Adult; Aged; Child; Endocrinology; Female; Follow-Up Studies; Humans; Hypogonadism; Hypothyroidism; Internal Medicine; Male; Medicine; Medicine & Public Health; Metabolic Diseases; Middle Aged; Nelson Syndrome - pathology; Nelson Syndrome - surgery; Original Article; Patients; Pituitary; Pituitary Neoplasms - pathology; Pituitary Neoplasms - surgery; Radiation; Radiosurgery; Radiosurgery - methods; Retrospective Studies; Treatment Outcome; Tumors; Visual perception; Young Adult
• ISSN: 1720-8386; 0391-4097; 1720-8386
• DOI: 10.1007/s40618-021-01531-6

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.