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.
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.
Authors: R Nocente; Marinis L De; A Mancini; A Bianchi; R Bellantone; L Lauriola; M Costanzo; Crea C De; G Gasbarrini; Silveri N Gentiloni Journal: J Endocrinol Invest Date: 2002-03 Impact factor: 4.256
Authors: John R Porterfield; Geoffrey B Thompson; William F Young; John T Chow; Raymond S Fryrear; Jon A van Heerden; David R Farley; John L D Atkinson; Fredric B Meyer; Charles F Abboud; Todd B Nippoldt; Neena Natt; Dana Erickson; Adrian Vella; Paul C Carpenter; Melanie Richards; J Aidan Carney; Dirk Larson; Cathy Schleck; Marilyn Churchward; Clive S Grant Journal: World J Surg Date: 2008-05 Impact factor: 3.352