Format

Send to

Choose Destination
See comment in PubMed Commons below
Am J Surg Pathol. 2011 Sep;35(9):1311-26. doi: 10.1097/PAS.0b013e31821ec4ce.

Primary bimorphic adrenocortical disease: cause of hypercortisolism in McCune-Albright syndrome.

Author information

1
Department of Laboratory Medicine and Pathology, Division of Endocrinology, Diabetes, Nutrition and Metabolism, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. carney.aidan@mayo.edu

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.

PMID:
21836496
PMCID:
PMC4140081
DOI:
10.1097/PAS.0b013e31821ec4ce
[Indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Lippincott Williams & Wilkins Icon for PubMed Central
    Loading ...
    Support Center