pigmented nodular adrenocortical 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)