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Table 1 Diagnostic criteria for CNC by Stratakis CA in 2001 [3]

From: Carney complex: a case with thyroid follicular adenoma without a PRKAR1A mutation

Major Criteria
1. Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)
2. Myxoma (cutaneous and mucosal)
3. Cardiac myxoma
4. Breast myxomatosis or fat-suppressed magnetic resonance imaging findings suggestive of this diagnosis
5. PPNAD or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test
6. Acromegaly due to GH-producing adenoma
7. LCCSCT or characteristic calcification on testicular ultrasonography
8. Thyroid carcinoma or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient
9. Psammomatous melanotic schwannoma
10. Blue nevus, epithelioid blue nevus (multiple)
11. Breast ductal adenoma (multiple)
12. Osteochondromyxoma
Supplemental criteria
1. Affected first-degree relative
2. Inactivating mutation of the PRKAR1A gene
  1. PPNAD primary pigmented nodular adrenocortical disease; GH growth hormone; LCCSCT large-cell calcifying Sertoli cell tumor