Table 1: Diagnostic criteria for carney complex [modified from Correa R, et al. [12].
Major diagnostic criteria for CNC |
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Spotty skin pigmentation with typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)
Breast myxomatosis* or fat-suppressed magnetic resonance findings suggestive of this diagnosis
Primary pigmented nodular adrenocortical disease* or paradoxical positive response of urinary glucocorticosteroid excretion to dexamethasone administration during Liddle's test
Acromegaly as a result of a growth hormone (GH)-producing adenoma*
*Histological confirmation is required for these findings |
Large-cell calcifying Sertoli cell tumor (LCCSCT) Myxoma* (cutaneous and mucosal) Cardiac myxoma*
Thyroid carcinoma* or multiple, hypoechoic nodules on thyroid ultrasound in a child younger than 18 years
LCCSCT* or characteristic calcification on testicular ultrasound
Psammomatous melanotic schwannomas (PMS)*
Blue nevus, epithelioid blue nevus*
Breast ductal adenoma*
Osteochondromyxoma* |
Supplementary criteria Affected first-degree relative Inactivating pathogenic variant in PRKAR1A |