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A Clinicopathologic Correlation

James M. Swain, MD; Clive S. Grant, MD; Richard T. Schlinkert, MD; Geoffrey B. Thompson, MD; Jon A. vanHeerden, MD; Ricardo V. Lloyd, MD; William F. Young, MD

Arch Surg. 1998;133:541-546.

Objectives  To investigate the clinical presentation, laboratory findings, and pathologic characteristics of patients with corticotropin (ACTH)-independent macronodular adrenal hyperplasia.

Design  Retrospective review.

Setting  Academic medical center.

Patients  All patients with bilateral adrenocortical nodules associated with ACTH-independent hypercortisolism without clinicopathologic features of primary pigmented nodular adrenocortical disease with atrophic internodular adrenal cortex.

Main Outcome Measures  Compare and contrast our findings with those previously reported; assess response to adrenalectomy.

Results  Nine patients met the criteria for corticotropin-independent macronodular adrenal hyperplasia. All patients had biochemical evidence of Cushing syndrome, although repetitive testing was frequently required. As a result, the diagnosis was delayed from 1 to 20 years. In all patients, both the low- and high-dose dexamethasone suppression tests failed to suppress cortisol secretion. No patient had elevated ACTH levels, and following curative bilateral adrenalectomy, no patient subsequently developed Nelson syndrome, with follow-up ranging from 1 to 8.5 years. Unique histologic features were identified in all cases.

Conclusion  Amalgamating this series with other clinical reports plus basic research information, corticotropin-independent macronodular adrenal hyperplasia must be considered a separate and legitimate cause of Cushing syndrome.

From the Departments of Surgery, Mayo Clinic, Scottsdale, Ariz (Drs Swain and Schlinkert), and Mayo Clinic, Rochester, Minn (Drs Grant, Thompson, and vanHeerden), and Departments of Pathology (Dr Lloyd) and Internal Medicine (Dr Young), Mayo Clinic, Rochester.

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