Table 2.

Diagnostic criteria for Carney complex (CNC) (1, 6). A patient should meet (1) two major criteria or (2) one major and one supplemental criterion to establish the diagnosis of CNC

Clinical manifestations	Prevalence (%)
Major criteria
Spotty skin pigmentation with typical distribution
Lentigines	70–80
Café-au-lait macules	Rare
Cutaneous and mucosal myxoma	30–55
Cardiac myxoma	20–40
Primary pigmented nodular adrenocortical disease (PPNAD) and/or paradoxical increase in urinary glucocorticoid excretion in response to dexamethasone administration during Liddle’s test	25–60
Acromegaly due to growth hormone-producing pituitary adenoma	10–12
Large-cell calcifying Sertoli cell tumor	41–70
Thyroid carcinoma (papillary or follicular type)	Up to 10
Psammomatous melanotic schwannomas	Up to 10
Blue nevus, epithelioid blue nevus	40
Breast ductal adenoma (multiple)	14–25
Osteochondromyxoma	Rare
Supplemental criteria
Affected first-degree relative
Inactivating mutation of the PRKAR1A gene
Activating pathogenic variants of PRKACA and PRKACB