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. 2019 Mar 21;2019:18-0150. doi: 10.1530/EDM-18-0150

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