Primary adrenocortical hyperplasia | ||||||
---|---|---|---|---|---|---|
Macronodular hyperplasia | Micronodular hyperplasia | |||||
Adrenocortical adenoma | Adrenocortical carcinoma | BMAH | c-BMAH | PPNAD | MAD | |
Frequency | ∼55% | ∼35% | Estimated 10% | |||
Age | All ages | All ages; familial cases in childhood | Fifth to sixth decade | Early childhood | Childhood or early adulthood | |
Clinical presentation | Mild-to-severe Cushing | Moderate-to-severe Cushing with rapid onset and possible virilisation | Mild Cushing with insidious onset | Moderate-to-severe Cushing | Moderate-to-severe Cushing | |
Biochemical features | Negative Liddle's test | Negative Liddle's test | Negative Liddle's test | Negative Liddle's test | Paradoxical cortisol response in Liddle's test | Negative Liddle's test |
Radiological features | Solitary mass, often <4 cm, defined margins, homogeneous, <10 HFU, absolute washout >60% | Solitary mass, often >4 cm, irregular margins, heterogenous, >10 HFU, absolute washout <60% | Marked bilateral adrenal enlargement with multiple large non-pigmented nodules (1–5 cm) | Normal or small size adrenal glands with occasional small nodules (<1 cm) |
BMAH, primary bilateral macronodular adrenocortical hyperplasia; c-BMAH, childhood BMAH; HFU, Hounsfield unit; MAD, non-pigmented micronodular adrenocortical disease; PPNAD, primary pigmented nodular adrenocortical disease.