Role of adrenalectomy in recurrent Cushing's disease

Abstract

Background: Cushing's disease is a pituitary-dependent type of Cushing's syndrome. Treatment consists of pituitary surgery or radiotherapy, but the recurrence rate at 10 years is as high as 40%. Adrenalectomy is considered an effective treatment to refractory Cushing's disease. The objective of this study was to examine the efficacy of laparoscopic adrenalectomy and open adrenalectomy in Cushing's disease, focusing on reversing the sequelae of hypercortisolism and improving patients' quality of life.

Methods: Forty-three patients (29 women, 14 men) with recurrent Cushing's disease after transsphenoidal operation underwent laparoscopic (n = 32) or open (n = 11) adrenalectomy from 2000 to 2008. Surgical results were evaluated for all the 43 patients. Patients completed a follow-up survey, including the short-form 36-item (SF-36) health survey.

Results: All the 43 patients achieved clinical reversal of hypercortisolism after adrenalectomy. Time to symptom resolution varied from a few weeks to up to 3 years. Most physical changes had resolved by a mean of 8 months after surgery. These conditions were not significantly different between the laparoscopy and open groups. Median length of hospital stay was shorter in the laparoscopy group (4 vs. 9 days; P < 0.001). Median follow-up was 48.5 months. Of the 34 (79%) patients available for follow-up, 22 (65%) had adrenocorticotropic hormone levels > 200 ng/ml and 6 (27%) had clinical Nelson syndrome. Four patients died by 75 months after surgery. Using SF-36, 30 (88%) patients reported they felt their health status was good to excellent compared with 1 year before adrenalectomy; however, they showed significantly lower scores in all the 8 SF-36 parameters compared with the general population. No significant difference emerged in SF-36 scores between the laparoscopy and open groups.

Conclusions: Adrenalectomy showed high survival and clinical benefits in recurrent Cushing's disease patients. Despite patient-reported improvement in health after adrenalectomy, patients continue to experience poor health status compared with the general population.