| 1 - Cutaneous lentiginosis with typical distribution (lips, conjunctiva, mucous membranes) |
| 2 - Myxoma (cutaneous and mucosal) or cardiac myxoma |
| 3 - Mammary myxomatosis or MRI findings suggestive of the diagnosis |
| 4 - Pigmented primary nodular disease or paradoxical increase in the excretion of urinary glucocorticoids after dexamethasone administration |
| 5 - Acromegaly associated with pituitary adenoma producing GH |
| 6 - Testicular tumor of large calcified sertoli cells or presence of calcifications in testicular ultrasound |
| 7 - Thyroid carcinoma or presence of multiple hypoechoic nodules in prepubertal thyroid ultrasound |
| 8 - Psammomatous melanocytic schwannomas |
| 9 - Blue nevus, multiple epithelioid blue nevus |
| 10 - Multiple ductal mammary adenomas |
| 11 - Osteochondromyoma |
| Supplementary Criteria |
| 1 - Affected family member |
| 2 - Presence of inactivating mutations of the PRKAR1A gene |
| 3 - Activating variants of the PRKACA gene or PRKACB gene |
