From Lewis S. Blevins, Jr. MD, – I’ve taken care of a patient for a number of years. She has had central hypothyroidism and growth and deficiency presumed due to a partial empty sella.
Recently, we did a follow up visit. She looked markedly Cushingoid. I indicated that her physicians were probably giving her too many steroids. She stated that she wasn’t taking any steroids!
During the visit I reviewed the chart and saw that she had a number of ACTH levels that were in the normal range but had periodic elevations in serum cortisol for a few years. A provider had recently seen a low ACTH and suspected that she might’ve developed additional pituitary deficits so she underwent an ACTH stimulation test. Her baseline cortisol, when the ACTH was undetectable, was in the 30s and her peak response was in the 60s.
So, how does one make sense of this data? First of all, any patient who looks Cushingoid should be asked whether they’re taking steroids. If not, they deserve a work up. Exogenous steroids are the most common cause of a Cushingoid appearance in people out there in the real world. If you see someone in the mall, for example, it’s more likely that they have been taking steroids than they would have a pituitary or adrenal disorder. The same is true of the ACTH level. If it’s low, the most likely cause is exogenous steroids rather than excessive endogenous cortisol production. While exogenous steroids can cross react in the cortisol essay, most of those essays are pretty sensitive, and the fact that her cortisol level was elevated, and she had a robust stimulation, rules out central adrenal insufficiency and suggests a syndrome of cortisol excess.
When ACTH levels are undetectable, or under 15 or so, we suspect primary adrenal disease as the cause of any apparent hypercortisolism or Cushingoid appearance. So, I told my patient we needed to do an adrenal CT scan. She indicated that she’d had a CT scan for a complicated kidney stone and they told her she had an adrenal problem and to review the studies with me. The next day, we obtained her abdominal CT scan and reviewed it. Here are the findings.
Figure one shows a 4.7 cm right adrenal adenoma. The lesion displaces or crunches the entire right adrenal gland. Figure 2 shows a synchronous 2.5 cm left adrenal adenoma.
What might this person have? First off, either one or both of these lesions could be secreting cortisol. It could be the big one or the small one. It’s most likely the big one. However, any adrenal tumor over 2 cm can cause absolute hypercortisolism. This patient might have a cortisol producing lesion on one side and a non-functioning lesion on the other. Or, the patient has bilateral macro modular hyperplasia. This would be rather unusual since there’s only one nodule in each adrenal gland. But I’ve seen it before.
The next step in the evaluation is to do a 24 hour urine free cortisol excretion rate to determine the magnitude of hypercortisolism. This is important because this patient might need medical therapy to control cortisol levels while she undergoes treatment for a complicated kidney stone. By the way, kidney stones can happen inpatient with Cushing’s so that might be related to the underlying problem.
On the other hand, the patient probably needs to have adrenal vein sampling to determine which of these two lesions is producing cortisol. Then, it could be surgically removed. Then, if both adrenal gland are secreting excess cortisol, the patient would be treated either medically or with a bilateral adrenalectomy.
One other potential approach is to remove the largest lesion, as lesions of about 5 cm have a 40% likelihood of being malignant, and it probably should come out anyways. Then, reassess the cortisol secretary dynamics to determine whether the patient has been rendered disease free or needs medication, or should have the remaining adrenal gland removed.
I will be discussing potential management strategies with our endocrine surgeon in the near future and we will arrive at a joint decision on how best to proceed. Teamwork is essential for patients with neoplastic adrenal disorders, and especially when there is evidence of cortisol, androgen, or aldosterone excess.
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