Table 3 Diagnostic criteria for Carney complex

Major criteria

Skin pigmentation disorders

  1. Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal, and penile mucosa)

  2. Blue nevus, epithelioid blue nevus (multiple)

Myxomas

  1. Myxoma (cutaneous and mucosal)

  2. Cardiac myxoma

  3. Breast myxomatosis or fat-suppressed magnetic resonance imaging findings suggestive of this diagnosis

  4. Osteochondromyxoma

Endocrine tumors/overactivity

  1. PPNAD or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test

  2. Acromegaly due to GH-producing adenoma

  3. LCCSCT or characteristic calcification on testicular ultrasonography

  4. Thyroid carcinoma or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient

  5. Psammomatous melanotic schwannoma

  6. Breast ductal adenoma (multiple)

Supplemental criteria

1. Affected first-degree relative

2. Inactivating mutation of the PRKAR1A gene