Primary bimorphic adrenocortical disease: Cause of hypercortisolism in mccune-albright syndrome

J. Aidan Carney, William F. Young, Constantine A. Stratakis

Research output: Contribution to journalArticlepeer-review

53 Scopus citations

Abstract

McCune-Albright syndrome (polyostotic fibrous dysplasia, café-au-lait skin spots, and precocious puberty) is a genetically mosaic disorder with populations of mutant and normal cells in affected organs. Cushing syndrome, a rare feature of the condition, usually affects infants and is the result of corticotropin-independent primary bilateral adrenal disease, usually interpreted as nodular adrenocortical hyperplasia. In this study of 9 patients with Cushing syndrome and McCune-Albright syndrome, light microscopy revealed a characteristic bimorphic pattern of diffuse and nodular hyperplasia and a distinctive form of cortical atrophy with apparent zona glomerulosa hyperplasia in 8 patients, all very young. The pattern could be explained by the presence of a mosaic distribution of mutant and normal cells in the adrenal glands. The findings are different from those in inherited or other forms of genetically caused Cushing syndrome. The ninth patient, aged 17 years, had an adrenal adenoma and diffuse cortical hyperplasia in each adrenal gland.

Original languageEnglish (US)
Pages (from-to)1311-1326
Number of pages16
JournalAmerican Journal of Surgical Pathology
Volume35
Issue number9
DOIs
StatePublished - Sep 2011

Keywords

  • Cushing syndrome
  • GNAS1 mutation
  • McCune-Albright syndrome
  • adrenal cortical atrophy
  • adrenal cortical hyperplasia

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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