Original Publication Date: 1 September, 2014
Publication / Source: International Journal of Endocrine Oncology
Authors: Hervé Lefebvre, Gaëtan Prévost, Estelle Louiset
Cushing’s syndrome is classified into corticotropin-dependent and corticotropin-independent (i.e., primary adrenal) diseases. Bilateral adrenal hyperplasias, either micronodular or macronodular, represent a rare cause of primary adrenal Cushing’s syndrome, which occurs in less than 2% of all cases. Micronodular pigmented and nonpigmented hyperplasias are genetic conditions, which, in the great majority of cases, are the consequence of PRKAR1A and PDE11A (or PDE8B) gene-inactivating mutations.