CORTICOTROPIN-INDEPENDENT MACRONODULAR ADRENAL-HYPERPLASIA - A CLINICOPATHOLOGICAL CORRELATION

Objectives: To investigate the clinical presentation, laboratory findi ngs, and pathologic characteristics of patients with corticotropin (AC TH)-independent macronodular adrenal hyperplasia. Design: Retrospectiv e review. Setting: Academic medical center: Patients: All patients wit h bilateral adrenocortical nodules associated with ACTH-independent hy percortisolism without clinicopathologic features of primary pigmented nodular adrenocortical disease with atrophic internodular adrenal cor tex. Main Outcome Measures: Compare and contrast our findings with tho se previously reported; assess response to adrenalectomy. Results: Nin e patients met the criteria for corticotropin-independent macronodular adrenal hyperplasia. All patients had biochemical evidence of Gushing syndrome, although repetitive testing was frequently required. As a r esult, 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 de veloped Nelson syndrome, with follow-up ranging from 1 to 8.5 years. U nique histologic features were identified in all cases. Conclusion: Am algamating this series with other clinical reports plus basic research information, corticotropin-independent macronodular adrenal hyperplas ia must be considered a separate and legitimate cause of Gushing syndr ome.