Treatment Options for Cushing Disease After Unsuccessful Transsphenoidal Surgery

James K. Liu, M.D.; Maria Fleseriu, M.D.; Johnny B. Delashaw Jr., M.D.; Ivan S. Ciric, M.D.; William T. Couldwell, M.D., Ph.D.


Neurosurg Focus. 2007;23(3):E8 

In This Article

Bilateral Adrenalectomy

Bilateral adrenalectomy is a safe, effective, and definitive treatment for patients with refractory Cushing disease who have undergone multiple treatments unsuccessfully or when immediate reversal of hypercortisolemia is needed. Recently, this operation has been performed via a minimally invasive laparoscopic approach rather than by a traditional open approach because the minimally invasive approach results in fewer complications and a shorter inpatient hospital stay. A recent series of 12 patients with Cushing disease who had not attained remission using previous treatments (such as transsphenoidal surgery, radiotherapy, and medical therapy) showed that laparoscopic adrenalectomy was successful in resolving several signs and symptoms such as proximal myopathy, hirsutism, emotional lability, and weight loss.[63] There was also an improvement in glucose tolerance and blood pressure control in all of these patients.

Patients undergoing bilateral adrenalectomy will require lifelong mineralocorticoid and glucocorticoid replacement, but their overall quality of life is improved when compared with their health preoperatively.[29,61] One major concern after adrenalectomy is the development of Nelson syndrome in patients with ACTH-secreting pituitary adenomas. Nelson syndrome is characterized by elevated serum ACTH levels, hyperpigmentation, and progressively enlarging pituitary tumors that are often invasive and, in rare cases, may develop into pituitary carcinomas.[35,48,49] This incidence has been reported to be between 8 and 38% in some series, with corticotroph tumor progression as high as 40% over 20 years.[4] Kemink and colleagues[34] found that a predictive factor for developing Nelson syndrome was undergoing bilateral adrenalectomy at an earlier age. The incidence of Nelson syndrome after the treatment of Cushing disease is higher in children than in adults.[60] Use of prophylactic pituitary radiation therapy to reduce the risk of developing Nelson syndrome is still debatable: some investigators have shown good results,[32] whereas others have shown no significant difference.[58]


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