A Patient With Preclinical Cushing's Syndrome and Excessive DHEA-S Secretion Having Unilateral Adrenal Carcinoma and Contralateral Adenoma.

Abstract

Abstract. We report a case of preclinical Cushing's syndrome in a 54-year-old male associated with bilateral adrenocortical tumours. Physical findings and general laboratory data were unremarkable except for mild hypertension (158/90 mmHg) and impaired glucose tolerance. Endocrinological evaluation revealed the presence of autonomous cortisol secretion including unsuppressible serum cortisol by 8 mg dexamethasone test (11,ug/dl), high serum DHEA-S (3580 ng/ml, normal: 400-3500) and increased urinary 17-KS excretion (31.0-35.8 mg/day, normal: 5.8-21.3). CT scan demonstrated the presence of tumours in both adrenals and bilateral adrenalectomy was subsequently performed. Histological examination of the resected specimens revealed an adrenocortical carcinoma on the right side and an adenoma on the left side with noticeable cortical atrophy in non-neoplastic adrenals. Immunohistochemical study of steroidogenic enzymes demonstrated that all the steroidogenic enzymes involved in cortisol biosynthesis were expressed in both right and left adrenal tumours. Enzymatic activities of 21, f3-hydroxylases were detected in both right and left adrenals except for the absence of 11 f3-hydroxylase activity in the left adrenal adenoma.Results of in vitro tissue steroidogenesis examined in short-term tissue culture of the specimens revealed no significant differences between carcinoma and adenoma in cortisol production, but the production of adrenal androgens in carcinoma was significantly higher than that in adenoma, which may indicate the importance of evaluating adrenal androgen levels in patients with adrenocortical neoplasms.

A Case of Estrogen-secreting Adrenocortical Carcinoma with Subclinical Cushing's Syndrome

“…Autonomous cortisol production without clinically overt Cushing's syndrome, termed as subclinical Cushing's syndrome, accounts for 5-12% of adrenal incidentaloma [14,[22][23][24]. However, ACC associated with subclinical Cushing's syndrome has rarely been reported thus far [25,26]. The diagnosis of subclinical Cushing's syndrome in the present case was made by the lack of Cushingoid feature and the following endocrine data; 1) suppression of plasma ACTH; 2) lack of circadian rhythm of cortisol; 3) no response of plasma ACTH and cortisol to CRH stimulation; 4) nonsuppressibility of cortisol to low-and high-dose (1, 2, 8 mg) dexamethasone, and 5) postoperative adrenal insufficiency necessiating cortisol replacement [25].…”

The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

“…It can be named as subclinical (or pre-clinical) Cushing's syndrome, subclinical autonomous glucocorticoid hypersecretion or subclinical hypercortisolism. Beside the Italian Society of Endocrinology study that has revealed four cases of subclinical Cushing's syndrome out of 47 ACC cases (8.5%), in the literature mostly only individual case presentations can be found [33][34][35][36].…”

Non polycystic ovary syndrome–related endocrine disorders associated with hirsutism

“…Androgen-secreting adrenal tumours, mostly carcinomas, are usually associated with the development of Cushingoid features and can be diagnosed as a typically large (> 6 cm) on ultrasound or adrenal computerized tomography. Increased production of adrenal androgens including DHEAS has been shown in patients with carcinoma than that in adenoma [51].…”