ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

Abstract

BackgroundAdrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome (CS) with bilateral cortisol-secreting adenomas has been rarely reported in the literatures. Precise recognition and management of this disorder constitute a challenge to clinicians due to the difficulty of exact location of the functional lesions.Case presentationWe herein report a new case of a Chinese female patient with a complaint of exertional dyspnea for over 10 years. ACTH-independent CS was diagnosed based on undetectable ACTH and unsuppressed cortisol levels by dexamethasone. Computed tomography (CT) scan indicated bilateral adrenal masses, and adrenal venous sampling (AVS) adjusted by plasma aldosterone revealed hypersecretion of cortisol from both adrenal glands. Bilateral cortisol-secreting adrenal adenomas were suspected and confirmed by the postoperative pathology in subsequent two-step bilateral laparoscopic adrenalectomy. The symptoms and signs of CS relieved after surgery with continuous glucocorticoid replacement.ConclusionsAVS adjusted by plasma aldosterone could be a useful technique in diagnosing ACTH-independent CS with bilateral adrenal adenomas prior to surgery. And the aldosterone ratio could be used to confirm the success of adrenal vein cannulation in this situation.

A case of adrenocortical adenoma harboring venous thrombus mimicking adrenal malignancy

“…In bilateral adrenal lesions with ACTH-independent CS, a size difference between left and right tumors has not been clearly documented in the literature [11]. In our patient, the right and left adrenal lesions showed distinct features on CT and MRI.…”

The Value of Adrenal Androgens for Correcting Cortisol Lateralization in Adrenal Venous Sampling in Patients with Normal Cortisol Secretion

“…To exclude the possibility of endogenous ACTH secretion interfering with the interpretation of autonomous cortisol secretion, all patients underwent AVS while receiving dexamethasone in Young's study [18]. However, not all of the studies followed this protocol, and the utility of the DST has been questioned [7, 8, 10]. It is well known that ACTH is fully suppressed in the majority of patients with ACTH-independent CS.…”

“…In PA patients, “cortisol-corrected” aldosterone ratios are compared to determine whether a unilateral source of aldosterone exists. Previous studies attempted to use epinephrine or aldosterone to correct for the side-to-side cortisol gradient [7–9]. Based on the data of Freel EM et al, in patients without pheochromocytoma, a gradient in catecholamine production between the right and left adrenal glands can be a normal finding.…”

“…Adrenal venous sampling (AVS) has been successfully used to lateralize the source of aldosterone hypersecretion in patients with primary aldosteronism [3, 4]. Several previous studies have reported using AVS for evaluation of cortisol-producing adrenal masses [1, 5–7]. Although it is helpful in some cases, this procedure is not successful in all cases [8–10].…”

Recent Advances in the Clinical Application of Adrenal Vein Sampling

“…In both cases with similar AVS results, distinct management was necessary to cure CS, which posed challenges in the interpretation of AVS results, particularly in the question of how to choose the reference hormone to calculate the cortisol to reference hormone ratios from the right and left adrenal veins and finally calculate the lateralization index in non-synchronous catheterization approaches, due to the rapid fluctuation in adrenal hormones during the AVS procedure. In 2018, Wei Jie el at., reported that aldosterone concentrations could be used as reference hormones to calculate the lateralization index ( 36 ). However, many factors can interfere with aldosterone concentrations.…”