Primary pigmented nodular adrenocortical disease: Unusual histology in children- A report of 3 cases

Authors : Reshma S Keskar, Ratnaprabha K Ghodke

DOI : 10.18231/j.ijpo.2022.038

Volume : 9

Issue : 2

Year : 2022

Page No : 165-168

­­­­­­­­­­­­­­­­­­Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of adrenocorticotropin independent Cushing Syndrome. Majority cases are diagnosed in second or third decade of life. Presentation of PPNAD in early childhood is very rare. It is characterized by adrenocorticotrophic hormone [ACTH] independent, hypersecretion of cortisol by multiple, pigmented nodules of hyperplastic adrenocortical cells. The histology varies with age, the pigmentation increasing with age. More than ninety percent of reported cases of PPNAD occur as one of the manifestation of Carney’s complex.
 

Keywords: PPNAD, Cushing Syndrome, Cortisol, ACTH independent.

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