ACTH-independent Cushing’s syndrome (CS) is typically caused by autonomous cortisol secretion from a single adrenal adenoma or carcinoma. The diagnosis and subsequent management of such patients can be complicated by the discovery of bilateral adrenal lesions on imaging; each lesion may be a non-functioning incidentaloma or a source of hypercortisolism. In this setting, adrenal vein sampling (AVS) may be utilised to determine lateralization of cortisol secretion. Here we discuss two such patients with contrasting adrenal pathology and post-operative management.
Patient #1 was an active 79-year-old female. Patient #2 was a 70-year-old retired male. Both presented with incidentally discovered adrenal lesions and had Cushingoid features, including hypertension, proximal myopathy and predominantly abdominal weight gain. Each had a suppressed ACTH, low DHEA-sulphate and failed a 1mg dexamethasone suppression test.
Both patients underwent AVS. Patient #1 demonstrated lateralization to the side of the larger adrenal lesion. Patient #2’s results did not show lateralization. Successful adrenal vein catheterisation was confirmed in Patient #2 using plasma metanephrine concentrations1. Both patients underwent unilateral adrenalectomy, with Patient #2 planned for future contralateral adrenalectomy.
Patient #1 required post-operative glucocorticoid replacement and continues on hydrocortisone while remaining clinically well. Her histopathological diagnosis was adrenocortical adenoma. Patient #2 developed post-operative complications, including intra-abdominal collections and non-occlusive venous thromboses. He was given short-term stress glucocorticoid cover. His histopathological diagnosis was primary bilateral macronodular adrenal hyperplasia; he is planned for future contralateral adrenalectomy.
We conclude that the results of AVS assisted in clinical decision-making in these two patients. We will discuss the role and technique of AVS in patients with adrenal CS and bilateral adrenal lesions. We will also review the post-operative management and follow-up of such patients in the context of the published medical literature.