A Decrease in the Dose of Pegvisomant was Needed for the Treatment of Acromegaly after Adrenalectomy in a Patient with Coexisting Preclinical Cushing's Syndrome

Abstract

We herein describe the case of a 47-year-old woman with pre-clinical Cushing's syndrome caused by a left adrenal adenoma, which was diagnosed 6 years after trans-sphenoidal selective removal of a pituitary adenoma for acromegaly at age 35. The patient was started on bromocriptine and then somatostatin analogues after the surgery; however, since her serum insulin-like growth factor-1 (IGF-1) values remained above the age-adjusted normal range, the treatment for acromegaly was switched from somatostatin analogues to pegvisomant (10 mg daily), before a left laparoscopic adrenalectomy. After the subsequent adrenalectomy, the dose of pegvisomant could be reduced gradually to once every 4 days without any increase in the serum IGF-1 values. This is the first report describing the need for a different dose of pegvisomant for the treatment of acromegaly before and after adrenalectomy for pre-clinical Cushing's syndrome.

Poorly-controlled Acromegaly Accompanied by Subclinical Adrenal Cushing's Syndrome after Surgery for Multiple Endocrine Tumors

“…Subsequently, the authors followed nine of the 27 patients for one year and identified one patient with subclinical hypercortisolism who exhibited an increase in the size of the adrenal mass, similar to that noted in our case (8,9). To our knowledge, other than the above-mentioned case reported by Scaroni et al and our current case, only three cases of acromegaly accompanied by adrenocorticotropic hormone-independent subclinical Cushing's syndrome have been reported (10)(11)(12). No other forms of endocrinological neoplasia were observed in any of the previously reported four cases.…”

“…Previous clinical studies assessing the associations between adrenal gland tumors and acromegaly have reported correlations between a larger tumor size (11) and an increased prevalence of adenoma or hyperplasia (7,12) as well as acromegaly. However, it remains controversial whether there is a causal relationship between adrenal morphological alterations and the disease activity.…”

Dramatic Improvement of Diabetes Mellitus Following the Treatment of Coexisting Acromegaly and Cushing's Syndrome

“…The relationship between insulin resistance and diabetic status were previously described in two of the five patients with acromegaly and Cushing's syndrome (7,10). In one patient, adrenalectomy and pituitary surgery improved the patient's insulin resistance and glucose control state (10).…”

“…That patient, however, did not demonstrate marked alterations in the treatment of diabetes, such as the discontinuation of insulin or a dramatic improvement of glucose control. The second patient was not diabetic before left adrenalectomy (7). Reports on the other three patients with acromegaly and Cushing's syndrome did not mention the glucose control status (8,9,11).…”

“…To the best of our knowledge, only 5 such cases have been reported in the literature (7)(8)(9)(10)(11). We herein describe a 56-year-old woman with both acromegaly and a unilateral cortisol-producing adrenal adenoma who showed a dramatic improvement in glucose control following the treatment of her acromegaly and Cushing's syndrome.…”