Table 2.

Carney Complex (CNC) diagnostic criteria proposed by Stratakis, Kirschner, and Carney (2001)

Features	Present case
Diagnostic criteria
1. Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)	Absent
2. Myxoma (cutaneous and mucosal)a	Present (two cutaneous and two mucosal myxomas)
3. Cardiac myxomaa	Present
4. Breast myxomatosisa or fat-suppressed magnetic resonance imaging findings suggestive of this diagnosis	Absent
5. PPNADa or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test	Absent
6. Acromegaly due to GH-producing adenomaa	Present
7. LCCSCTa or characteristic calcification on testicular ultrasonography	Not applicable
8. Thyroid carcinomaa or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient	Absent
9. Psammomatous melanotic schwannomaa	Absent
10. Blue nevus, epithelioid blue nevus (multiple)a	Absent
11. Breast ductal adenoma (multiple)a	Absent
12. Osteochondromyxomaa	Absent
Supplemental criteria:
1. Affected first-degree relative	Absent
2. Inactivating mutation of the PRKAR1A gene	Not evaluated

To confirm a diagnosis of CNC, a patient must either: (1) exhibit two diagnostic features or (2) exhibit one diagnostic feature and one of the supplemental criteria [1]

^aMicroscopically confirmed. GH growth hormone, LCCSCT large cell-calcifying Sertoli cell tumor, PPNAD primary pigmented nodular adrenocortical disease