Bilateral adrenal lesions account for approximately 20% of all adrenal incidentalomas. Rarely, these lesions are associated with hypercortisolemia. In many cases, cortisol excess is subclinical, mild autosomal cortisol secretion (MACS), and patients do not present with overt Cushing’s syndrome. Patients with MACS are predisposed to hypertension and glucose intolerance and unilateral adrenalectomy of the larger adrenal gland is recommended as a treatment option on an individualized basis based on presence of above comorbidities, age, surgical risk and patient preferences. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare condition characterized by bilateral benign adrenal macronodules and accounts for one up to one-third of bilateral adrenal lesions and hypercortisolemia. The pathophysiology includes aberrantly expressed G-protein-coupled receptors (GPCRs) that are activated by hormones other than ACTH as well as germ line mutations in ARMC5 gene. Medical treatment for this condition can include targeted therapy for the aberrant receptors, although the success for such therapies has been limited so far. At this time, the optimal treatment for hypercortisolemia related to bilateral adrenal mass is not established. Current guidelines recommend against bilateral adrenalectomy in patients without overt Cushing’s syndrome. Unilateral adrenalectomy of the largest adrenal gland or the one with the biggest nodules is gaining support as the treatment option for patients with mild to moderate hypercortisolemia.

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Bilateral Adrenalectomy Versus Medical Management for Cushing’s Syndrome with Bilateral Adrenal Hyperplasia

  • Susan Sam

摘要

Bilateral adrenal lesions account for approximately 20% of all adrenal incidentalomas. Rarely, these lesions are associated with hypercortisolemia. In many cases, cortisol excess is subclinical, mild autosomal cortisol secretion (MACS), and patients do not present with overt Cushing’s syndrome. Patients with MACS are predisposed to hypertension and glucose intolerance and unilateral adrenalectomy of the larger adrenal gland is recommended as a treatment option on an individualized basis based on presence of above comorbidities, age, surgical risk and patient preferences. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare condition characterized by bilateral benign adrenal macronodules and accounts for one up to one-third of bilateral adrenal lesions and hypercortisolemia. The pathophysiology includes aberrantly expressed G-protein-coupled receptors (GPCRs) that are activated by hormones other than ACTH as well as germ line mutations in ARMC5 gene. Medical treatment for this condition can include targeted therapy for the aberrant receptors, although the success for such therapies has been limited so far. At this time, the optimal treatment for hypercortisolemia related to bilateral adrenal mass is not established. Current guidelines recommend against bilateral adrenalectomy in patients without overt Cushing’s syndrome. Unilateral adrenalectomy of the largest adrenal gland or the one with the biggest nodules is gaining support as the treatment option for patients with mild to moderate hypercortisolemia.