Key result
Three pediatric cases of primary pigmented nodular adrenocortical disease presented with ACTH-independent Cushing syndrome and unusual histology lacking characteristic lipofuscin pigmentation.
Case Report (n=3)
In young children, PPNAD may present with atypical histology lacking the characteristic lipofuscin pigment, which typically increases with age.
May complicate PPNAD diagnosis in young children; hypothesis-generating and leaves open need for age-specific criteria.
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.
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Keskar et al. (2022) conducted a case report in Primary pigmented nodular adrenocortical disease (PPNAD) (n=3). Bilateral adrenalectomy was evaluated. Three pediatric cases of primary pigmented nodular adrenocortical disease presented with ACTH-independent Cushing syndrome and unusual histology lacking characteristic lipofuscin pigmentation.
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