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pigmented nodular adrenocortical disease

PPNAD, primary pigmented micronodular adrenal disease

 

Association

-  Carney complex (Cushing disease,spotty cutaneous pigmentation, cutaneous and cardiac myxomas, growth hormone secreting pituitary adenomas, psammomatous melanotic schwannomas, multiple myxoid fibroadenomas of breast in females, large cell calcifying Sertoli tumors of testis in males)

Macroscopy

-  variable sized adrenal glands with multiple brown-black pigmented cortical nodules

Microscopy

-  sharply circumscribed nodules composed of eosinophilic lipid-poor cells similar to normal zone reticularis
-  growth pattern: trabecular
-  prominent lipofuscin deposits in zona fasciculata
-  focal necrosis
-  mitotic activity

Etiology

-  mutations in the PRKAR1A gene coding for the cyclic AMP-dependent protein kinase A (PKA) regulatory subunit 1A (#12424709#)


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