Cushing's syndrome due to a large adrenocortical adenoma with histological features simulating ACTH-independent macronodular adrenocortical hyperplasia

A 53-year-old woman presented with Cushing's syndrome resulting from an adrenocortical adenoma, 6.5 cm in diameter and 75 g in weight, which is larger than usual. Endocrinological data of this patient showed adrenocorticotropin (ACTH)-independent hypercortisolemia. A computed tomography scan of the adrenal glands revealed a single large and well-encapsulated tumor with an irregularly shaped area of calcification and loss of parenchyma on the left adrenal. The right adrenal gland was atrophic. Laparoscopic removal of the left adrenal tumor was performed. The tumor was lobulated and clearly encapsulated, and the non-neoplastic area of the left adrenal was atrophic without any nodularity. The histological analysis confirmed the diagnosis of adrenal adenoma. In addition, this adenoma displayed histopathological features in common with ACTH-independent macronodular adrenocortical hyperplasia (AIMAH), including clear cell predominance, a pattern of small compact cell nests in clear cell areas, and very long cord-like arrangement of small compact cells. In AIMAH, adrenals are extremely enlarged and are more massive than in any other subtype of Cushing's syndrome. The fact that the present adrenocortical adenoma was larger than those typical adenomas of Cushing's syndrome may reflect an AIMAH-type cellular composition of clear cell predominance and small compact cell nests.     

术前护理干预对ACTH-非依赖性双侧肾上腺大结节增生症腹腔镜手术患者负性情绪的影响

目的探讨护理干预减轻ACTH-非依赖性双侧。肾上腺大结节增生症（AIMAH）患者负性情绪的效果。方法对40例预行腹腔镜手术治疗的（AIMAH）患者,应用术前健康教育、心理干预等措施进行干预,采用焦虑自评量表（SAS）和抑郁自评量表（SDS）在干预前后进行心理评估,并测定睡眠时间。结果40例AIMAH患者入院初期均存在不同程度的消极情绪。实施护理干预后,患者SAS及SDS得分较干预前明显降低[（40．68±7．42）分比（51．56±10．16）分;（42．63±7．98）分比（58．64±11．14）分],干预前后比较,差异有统计学意义（t=4．212,P〈0．05）;实施护理干预后,患者睡眠时间延长f（6．96±0．42）h比（5．82±0．31）h]。干预前后比较,差异有统计学意义（t=3．305,P〈0．01）。结论对AIMAH患者实施有针对性的护理干预,可减轻患者焦虑、抑郁、恐惧等负性情绪,可提高患者的依从性,使其更好的配合治疗,促进其早日康复。     

Aberrant Expression of Hormone Receptors in Adrenal Cushing's Syndrome

In recent years, a novel understanding of the pathophysiology of adrenal Cushing's syndrome has emerged. The ectopic or aberrant expression of G-protein-coupled hormone receptors in the adrenal cortex was found to play a central role in the regulation of cortisol secretion in ACTH-independent macronodular adrenal hyperplasia (AIMAH) and in some unilateral adrenal adenomas. Various aberrant receptors, functionally coupled to steroidogenesis, have been reported: GIP, vasopressin, β-adrenergic, LH/hCG, and serotonin receptors have been best characterized, but angiotensin, leptin, glucagon, IL-1 and TSH receptors have also been described. The molecular mechanisms responsible for the aberrant expression of these receptors are currently unknown. One or many of these aberrant receptors are present in most cases of AIMAH and in some cases of adrenal adenomas with overt or sub-clinical secretion of cortisol. Clinical protocols to screen for such aberrant receptors have been developed and should be performed in all patients with AIMAH. The identification of such aberrant regulation of steroidogenesis in AIMAH provides the novel opportunity to treat some of these patients with pharmacological agents that either suppress the endogenous ligand or block the aberrant receptor, thus avoiding bilateral adrenalectomy. This work was supported by a grant from the Canadian Institutes of Health Research (MA-10339).     

ACTH-independant macronodular adrenal hyperplasia: imaging findings of a rare condition

Endogenous Cushing’s syndrome is a relatively rare disease. Most cases being ACTH-dependent, ACTH-independent Cushing’s syndrome (AICS) is an even rarer condition [15%–20%]. In more than 95% cases the cause of AICS is unilateral adrenal enlargement caused by adenoma or carcinoma. Bilateral adrenal disease is caused by primary pigmented nodular adrenal dysplasia (PPNAD) and ACTH-independent macro nodular hyperplasia (AIMAH). Only few case reports of the latter condition exist in the radiology literature, PPNAD being the commoner of two as the cause for AICS.