Corticotropin-independent macronodular adrenal hyperplasia: A clinicopathologic correlation

James M. Swain, Clive S. Grant, Richard T. Schlinkert, Geoffrey B. Thompson, Jon A. VanHeerden, Ricardo V. Lloyd, William Francis Young

Research output: Contribution to journalArticle

45 Citations (Scopus)

Abstract

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 highdose 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.

Original languageEnglish (US)
Pages (from-to)541-546
Number of pages6
JournalArchives of Surgery
Volume133
Issue number5
DOIs
StatePublished - May 1998

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Cushing Syndrome
Adrenocorticotropic Hormone
Adrenalectomy
Nelson Syndrome
Acth-Independent Macronodular Adrenal Hyperplasia
Delayed Diagnosis
Adrenal Cortex
Dexamethasone
Hydrocortisone
Outcome Assessment (Health Care)
Research

ASJC Scopus subject areas

  • Surgery

Cite this

Swain, J. M., Grant, C. S., Schlinkert, R. T., Thompson, G. B., VanHeerden, J. A., Lloyd, R. V., & Young, W. F. (1998). Corticotropin-independent macronodular adrenal hyperplasia: A clinicopathologic correlation. Archives of Surgery, 133(5), 541-546. https://doi.org/10.1001/archsurg.133.5.541

Corticotropin-independent macronodular adrenal hyperplasia : A clinicopathologic correlation. / Swain, James M.; Grant, Clive S.; Schlinkert, Richard T.; Thompson, Geoffrey B.; VanHeerden, Jon A.; Lloyd, Ricardo V.; Young, William Francis.

In: Archives of Surgery, Vol. 133, No. 5, 05.1998, p. 541-546.

Research output: Contribution to journalArticle

Swain, JM, Grant, CS, Schlinkert, RT, Thompson, GB, VanHeerden, JA, Lloyd, RV & Young, WF 1998, 'Corticotropin-independent macronodular adrenal hyperplasia: A clinicopathologic correlation', Archives of Surgery, vol. 133, no. 5, pp. 541-546. https://doi.org/10.1001/archsurg.133.5.541
Swain JM, Grant CS, Schlinkert RT, Thompson GB, VanHeerden JA, Lloyd RV et al. Corticotropin-independent macronodular adrenal hyperplasia: A clinicopathologic correlation. Archives of Surgery. 1998 May;133(5):541-546. https://doi.org/10.1001/archsurg.133.5.541
Swain, James M. ; Grant, Clive S. ; Schlinkert, Richard T. ; Thompson, Geoffrey B. ; VanHeerden, Jon A. ; Lloyd, Ricardo V. ; Young, William Francis. / Corticotropin-independent macronodular adrenal hyperplasia : A clinicopathologic correlation. In: Archives of Surgery. 1998 ; Vol. 133, No. 5. pp. 541-546.
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