Primary pigmented nodular adrenocortical disease: the original 4 cases revisited after 30 years for follow-up, new investigations, and molecular genetic findings.

Abstract	The original 4 patients with Cushing syndrome who underwent bilateral adrenalectomy for primary pigmented nodular adrenocortical disease were followed up for an average of 31 years to determine whether they or any of their primary relatives had developed Carney complex or its components. None had. Three of the patients were alive and well; the fourth had died of an unrelated condition. All the adrenal glands contained multiple small, black or brown cortical nodules, up to 4 mm in diameter. The extracapsular extension of the micronodules was limited to the immediate pericapsular adipose tissue and was not considered evidence of low-grade malignancy. Immunocytochemically, the nodules were positive for synaptophysin, inhibin-A, and melan A and negative for vimentin and CD56. Ki-67 antibody stained the cytoplasm of cells in the micronodules but not that of the atrophic cortical cells. The 4 patients had the PRKAR1A deletion that has been associated with the isolated form of primary pigmented nodular adrenocortical disease.
Authors	J Aidan Carney, Rossella Libé, Jérôme Bertherat, William F Young
Journal	The American journal of surgical pathology (Am J Surg Pathol) Vol. 38 Issue 9 Pg. 1266-73 (Sep 2014) ISSN: 1532-0979 [Electronic] United States
PMID	24805858 (Publication Type: Journal Article)
Chemical References	Biomarkers Cyclic AMP-Dependent Protein Kinase RIalpha Subunit PRKAR1A protein, human Dexamethasone Hydrocortisone
Topics	Adolescent Adrenal Cortex Diseases (genetics, metabolism, pathology, surgery) Adrenalectomy Biomarkers (analysis, urine) Carney Complex (genetics, metabolism, pathology) Child Cushing Syndrome (genetics, metabolism, pathology, surgery) Cyclic AMP-Dependent Protein Kinase RIalpha Subunit (genetics) Dexamethasone (administration & dosage) Female Follow-Up Studies Gene Deletion Genetic Predisposition to Disease Humans Hydrocortisone (urine) Immunohistochemistry Male Phenotype Polymerase Chain Reaction Predictive Value of Tests Time Factors Treatment Outcome Young Adult

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