Major Criteria

1) Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vagina or penile membranes)^a

2) Myxomas (cutaneous and mucosal)^a

3) Cardiac myxoma^a

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

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

6) Acromegaly due to GH-producting adenoma^a

7) LCCSCT^a or characteristic calcification on testicular ultrasonography

8) Thyroid carcinoma or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient

9) Psammomatous melanotic schwannomas^a

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

11) Breast ductal adenoma (multiple)^a

12) Osteochondromyxoma^a