Cardiac myxomas: A narrative review

Table 1 Diagnostic criteria for Carney complex

Diagnostic criteria
Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)
Myxoma (cutaneous and mucosal)1
Cardiac myxoma1
Breast myxomatosis1 or fat-suppressed magnetic resonance imaging findings suggestive of this diagnosis
Primary pigmented nodular adrenocortical disease1 or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test
Acromegaly due to growth hormone-producing adenoma1
Large cell calcifying Sertoli cell tumour1 or characteristic calcification on testicular ultrasonography
Thyroid carcinoma1 or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient
Psammomatous melanotic schwannoma1
Blue nevus, epithelioid blue nevus (multiple)1
Breast ductal adenoma (multiple)1
Osteochondromyxoma1
Supplemental criteria:
Affected first-degree relative
Inactivating mutation of thePRKAR1A gene

¹With histologic confirmation.