Primary bilateral adrenal nodular disease with Cushing's syndrome: varying aetiology

Abstract

Primary adrenal disorders contribute 20%â€"30% of patients with endogenous Cushing's syndrome. Most of the primary adrenal diseases are unilateral and include adenoma and adrenocortical carcinoma, whereas bilateral adrenal lesions are uncommon and include primary pigmented nodular adrenocortical disease, primary bilateral macronodular adrenocortical hyperplasia, isolated micronodular adrenocortical disease, bilateral adenomas or carcinomas, and rarely pituitary adrenocorticotropic hormone-dependent adrenal nodular disease. Cyclic adenosine monophosphate-dependent protein kinase A signalling is the major activator of cortisol secretion in primary adrenal nodular disorders. We report two cases of bilateral adrenal nodular disease with endogenous Cushing's syndrome, including one each of primary pigmented nodular adrenocortical disease and primary bilateral macronodular adrenocortical hyperplasia.

Keywords: adrenal disorders; endocrine cancer.

Figure 1

(A) Clinical picture of the patient with primary pigmented nodular adrenocortical disease. (B) Contrast-enhanced CT of the abdomen showing ‘string of beads’ appearance of the right adrenal gland. (C) Microphotograph showing large nodule comprising cells with abundant eosinophilic cytoplasm and compressed adrenal at periphery (H&E 10×). (D) Higher magnification showing abundant brownish pigment within the cytoplasm (H&E 20×).

Figure 2

(A) Clinical picture of the patient with primary bilateral macronodular adrenocortical hyperplasia (PBMAH). (B) Contrast-enhanced CT of the abdomen showing bilateral bulky adrenals suggestive of PBMAH and (C) showing multiple non-pigmented nodules, and nodular hyperplasia of compact and clear cells of the adrenal cortex with preservation of internodular tissue seen as eosinophilic areas suggestive of PBMAH.