Effect of stereotactic radiosurgery before bilateral adrenalectomy for Cushing's disease on the incidence of Nelson's syndrome

Clinical article

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Object

Nelson's syndrome (NS) is a significant and frequent risk for patients with Cushing's disease (CD) who undergo bilateral adrenalectomy. A recent study has shown tumor progression in 47% of patients at risk for NS. The authors sought to define the rate of NS in patients who were treated with Gamma Knife stereotactic radiosurgery (GK SRS) prior to bilateral adrenalectomy.

Methods

Consecutive patients with CD who were treated with GK SRS after pituitary surgery but before bilateral adrenalectomy were included. Serial MRI sequences were analyzed to evaluate for pituitary tumor growth. Clinical evaluations were performed to screen for NS. Follow-up for adrenocorticotropic hormone levels and hormone studies of other pituitary axes was performed.

Results

Twenty consecutive patients were followed with neuroimaging and clinically for a median of 5.4 years (range 0.6–12 years). One patient (5%) developed pituitary tumor growth consistent with NS 9 months after adrenalectomy. By Kaplan-Meier analysis, progression-free survival was 94.7% at 1, 3, and 7 years. No predisposing factors were identified for the tumor progression. Two patients developed new pituitary dysfunction and no patient developed cranial neuropathy or visual deficit after GK SRS.

Conclusions

These findings suggest that GK SRS not only serves a role as second-line therapy for CD, but that it also provides prophylaxis for NS when used before bilateral adrenalectomy.

Abbreviations used in this paper:ACTH = adrenocorticotropic hormone; CD = Cushing's disease; GK SRS = Gamma Knife stereotactic radiosurgery; NS = Nelson's syndrome.

Article Information

Address correspondence to: Mary Lee Vance, M.D., Department of Medicine, University of Virginia Health System, Charlottesville, VA 22908. email: mlv@virginia.edu.

Please include this information when citing this paper: published online August 16, 2013; DOI: 10.3171/2013.7.JNS13389.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Postoperative MRI study of the sella obtained in 1 patient 9 months after bilateral adrenalectomy (left) demonstrating new tumor growth (arrowheads) corresponding with elevated ACTH levels. After targeted GK SRS for the lesion (25 Gy, 5 isocenters) and initiation of cabergoline therapy, the tumor was no longer appreciated on pituitary imaging obtained during 8.5 years of follow-up (right).

  • View in gallery

    Kaplan-Meier analysis demonstrates infrequent tumor progression after bilateral adrenalectomy in patients previously treated with GK SRS. The proportion of patients without tumor progression at 1, 3, 7, and 10 years of follow-up was 94.7% at each time point (number of patients at risk: 18, 12, 5, and 1, respectively). Dashed lines indicate 95% confidence intervals.

  • View in gallery

    Graphic representation of initial (prior to adrenalectomy) and maximum ACTH levels during follow-up in 18 patients. The ACTH levels typically increased during follow-up. Eleven (61%) and 4 (22%) patients developed maximum ACTH levels greater than 200 and 1000 pg/ml, respectively. The patient who developed NS (dashed line) did not have ACTH testing before adrenalectomy, but developed rising ACTH levels and tumor growth after treatment, which responded to repeat GK SRS (asterisk).

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