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 |