Coexisting acromegaly and a unilateral cortisol-producing adrenal adenoma: a possible variant of multiple endocrine neoplasia type I

Abstract

An unusual case of coexisting acromegaly and Cushing's syndrome is reported in a 34-yr-old female. There was no biochemical or morphological evidence to suggest the presence of other endocrinopathies. She did not have any family history to suggest a hereditary tendency to endocrine disorders. Her acromegaly and Cushing's syndrome were proven to be due to a pituitary somatotroph adenoma and a cortisol-producing adenoma in the right adrenocortex, respectively. Surgical removal of both tumors led to a marked biochemical improvement of the two endocrinopathies. To account for the simultaneous occurrence of the two endocrine tumors, at least two endocrine syndromes may be considered. One of them is Carney's complex. However, Cushing's syndrome in this complex is unexceptionally due to primary pigmented nodular adrenocortical disease, differing from the adrenal pathology of our patient. In addition, a lack in this case of any other characteristic suggestive of this syndrome appears to speak against this possibility. A second possibility is multiple endocrine neoplasia type 1. The absence of a parathyroid or pancreatic islet cell tumor does not strongly support this possibility, but adrenocortical lesions are not rare in this syndrome although they are only rarely functional. However, existence of similar case reports, although very few, in the literature leaves the possibility that she represents another rare variant of sporadic multiple endocrine neoplasia type I 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.…”

A Case of Acromegaly Accompanied by Adrenal Preclinical Cushing's Syndrome.

“…A recent review of the English literature revealed that there were only two reports of the coexistence of acromegaly and ACTHindependent Cushing's syndrome. One was a case of acromegaly with Cushing's syndrome due to bilateral nodular adrenocortical hyperplasia [16], and the other was due to a unilateral adrenocortical adenoma [17]. The latter case, which had been previously reported by us, was a case of preclinical Cushing's syndrome [17].…”

“…One was a case of acromegaly with Cushing's syndrome due to bilateral nodular adrenocortical hyperplasia [16], and the other was due to a unilateral adrenocortical adenoma [17]. The latter case, which had been previously reported by us, was a case of preclinical Cushing's syndrome [17]. After adrenalectomy, patients with overt Cushing's syndrome usually suffer from adrenal insufficiency requiring glucocorticoid replacement therapy for 9-33 months [13], but in our patient, glucocorticoid replacement therapy could be withdrawn as early as 8 weeks after adrenalectomy.…”

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

“…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.…”