The accelerated development of Cushing's syndrome in a patient with known adrenal tumors introduces the differential diagnosis of adrenocortical carcinoma. Adrenal tumors, adrenal hyperplasia, and primary pigmented nodular adrenocortical disease can all lead to Cushing's syndrome, although the presentation of these conditions tends to be gradual.
Adrenocortical carcinoma is very rare, with an annual incidence of approximately two cases per 1 million population.5 Of the 60% of adrenocortical carcinomas that are functional (i.e., hormone-secreting), 45% secrete both androgens and glucocorticoids; the rest secrete glucocorticoids alone (45%), androgens alone (10%), or, rarely, aldosterone (<1%).2 The clinical manifestations of hormone excess in adrenocortical carcinomas are rapidly progressive, as in this case.
Imaging can be helpful in differentiating benign from malignant adrenal masses. Low attenuation on a CT scan obtained without the administration of contrast material suggests a substantial lipid content that is most consistent with adrenal adenoma. Adrenal adenomas also tend to wash out at least 60% of the contrast material within 15 minutes after contrast administration.6 Adrenal cancers generally have an irregular appearance, high attenuation, and rapid growth. None of these are apparent in this case, and the relative stability of this patient's tumors in recent years makes a diagnosis of adrenocortical cancer unlikely. It is more likely that the recent bilateral adrenal enlargement in this case is due to either adrenal metastases or adrenal hyperplasia in response to corticotropin than to the development of hormonally active adrenal nodules.
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