Table 1 Diagnostic criteria for CNC (from [5] with written permission)

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

• Myxoma (cutaneous and mucosal)^a

• Cardiac myxoma^a

• Breast myxomatosis^a or fat-suppressed MRI findings suggestive of this diagnosis

• PPNAD^a or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test

• Acromegaly due to GH-producing adenoma^a

• LCCSCT^a or characteristic calcification on testicular ultrasonography

• Thyroid carcinoma (at any age)^a or multiple, hypoechoic nodules on thyroid ultrasonography in a prepubertal child

• Psammomatous melanotic schwannoma^a

• Blue nevus, epithelioid blue nevus (multiple)^a

• Breast ductal adenoma (multiple)^a

• Osteochondromyxoma