Abstract
It has been well established that, in the human adrenal gland, cortisol secretion is not only controlled by circulating corticotropin but is also influenced by a wide variety of bioactive signals, including conventional neurotransmitters and neuropeptides, released within the cortex by various cell types such as chromaffin cells, neurons, cells of the immune system, adipocytes, and endothelial cells. These different types of cells are present in bilateral macronodular adrenal hyperplasia (BMAH), a rare etiology of primary adrenal Cushing’s syndrome, where they appear intermingled with adrenocortical cells in the hyperplastic cortex. In addition, the genetic events, which cause the disease, favor abnormal adrenal differentiation that results in illicit expression of paracrine regulatory factors and their receptors in adrenocortical cells. All these defects constitute the molecular basis for aberrant autocrine/paracrine regulatory mechanisms, which are likely to play a role in the pathophysiology of BMAH-associated hypercortisolism. The present review summarizes the current knowledge on this topic as well as the therapeutic perspectives offered by this new pathophysiological concept.
Highlights
Chronic hypercortisolism results in a series of symptoms, including central obesity, skin changes, and arterial hypertension, known as Cushing’s syndrome
This unequal repartition of steroidogenic enzymes among adrenocortical cells may result in relatively inefficient steroidogenesis, likely explaining the discrepancy between the major enlargement of the adrenal glands and the moderate intensity of hypercortisolism generally observed in patients with Bilateral macronodular adrenal hyperplasia (BMAH)
Adrenal vein sampling demonstrated a significant ACTH concentration gradient between the adrenal versus peripheral veins as well as inferior petrosal sinus in one of the two patients [24, 36]. All these results suggested that ACTH produced by intraadrenal gonadal-like cells may stimulate cortisol secretion in BMAH tissues, supplying pituitary ACTH, which is suppressed by cortisol excess
Summary
Chronic hypercortisolism results in a series of symptoms, including central obesity, skin changes, and arterial hypertension, known as Cushing’s syndrome. Bilateral macronodular adrenal hyperplasia (BMAH) is a rare cause of primary adrenal hypercortisolism representing
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