Elsevier

Surgery

Volume 141, Issue 2, February 2007, Pages 147-152
Surgery

American Association of Endocrine Surgeon
Nelson’s syndrome after bilateral adrenalectomy for Cushing’s disease

Presented at the 27th Annual Meeting of the American Association of Endocrine Surgeons, New York, New York, May, 2006.
https://doi.org/10.1016/j.surg.2006.12.003Get rights and content

Background

Presentation and outcome of Nelson’s syndrome after bilateral adrenalectomy is variable.

Methods

Clinical records of 39 patients who underwent bilateral adrenalectomy for primary or recurrent Cushing’s disease during a 15-year period were analyzed for frequency and evolution of Nelson’s syndrome.

Results

The study included 32 females and 7 males with a mean age of 31 years; 20 patients had a hypophysectomy as the initial procedure, and 19 had an adrenalectomy. Of the group, 17 patients received prophylactic radiation therapy to the pituitary gland. A total of 11 patients, none of whom had received prophylactic radiation therapy, developed Nelson’s syndrome (determined by skin hyperpigmentation, elevated serum ACTH levels, and enlargement of a previous pituitary tumor or development of a new tumor in patients with no previous pituitary abnormality) over a mean follow-up period of 53 months. Treatment for Nelson’s syndrome included valproic acid, radiation therapy, and hypophysectomy as monotherapy or combined therapy. Of the remaining 28 patients, 10 (7 without prophylactic radio therapy) developed skin hyperpigmentation and increased ACTH levels without a tumor.

Conclusions

Nelson’s syndrome is a frequent complication after bilateral adrenalectomy in the absence of prophylactic radiotherapy (28%). The syndrome can be successfully controlled by medical treatment and or radiotherapy; patients rarely require hypophysectomy.

Section snippets

Patients and methods

Clinical records of patients subjected to bilateral adrenalectomy for Cushing’s disease at our institution from March 1990 to June 2005 were reviewed for skin hyperpigmentation, ACTH levels, growth of a previous or new pituitary tumor, details of the adrenal surgery, outcome of Cushing symptoms, episodes of adrenal insufficiency and radiotherapy. The latter was considered prophylactic when given prior to or immediately after bilateral adrenalectomy and before development of Nelson’s syndrome,

Results

Our study included 39 patients: 32 females (82%) and 7 males (18%), with a diagnosis of primary or recurrent Cushing’s disease and a minimum follow-up of 12 months. Mean age was 31 years (range, 15 to 58).

The first 11 adrenalectomies were performed using the open posterior approach; the subsequent 28 were laparoscopic (lateral intraperitoneal) with 1 conversion due to bleeding and excessive adipose tissue. Mean operative time was 229 min (range, 180 to 270) for the open procedures and 298 min

Discussion

Transsphenoidal hypophysectomy has proven to be the most cost-effective treatment for patients with Cushing’s disease and is the preferred initial therapy worldwide. Pituitary surgery has an average success rate between 75% and 80%, and a recurrence rate from 7% to 12% in successfully operated patients.6 Although bilateral adrenalectomy is an effective treatment for Cushing’s disease, it is weighed down by significant perioperative mortality and morbidity rates, by the risk of developing

References (14)

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    Such patients also have a higher chance of relapse following TSS (7–12% of patients with Cushing's disease treated with TSS have a relapse within one year following the procedure (Lanzi et al., 1998)) and subsequent need for TBA. Consistent with this hypothesis is the observation that compared with patients with less aggressive forms of pituitary tumor, patients with invasive corticotropinomas at the time of TSS are also at greater risk of subsequently developing Nelson syndrome at an earlier stage following TBA (Nagesser et al., 2000; Gil-Cardenas et al., 2007). The de novo development of a new corticotropinoma that is clonally distinct from the original tumor is also possible in some patients, although there is little evidence to support this alternative theory.

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