Table 3.

Conditions to be considered in the differential diagnosis of CNC per tissue manifestation

Organ	Related Disorders
Heart (cardiac myxomas)	Sporadic myxomas Adults: most common type of cardiac tumor Children: approximately 30% of cardiac tumor Familial myxomas due to mutation of a protein of the myosin family
Skin (lentigines)	Familial lentiginosis, Peutz-Jeghers syndrome, LEOPARD syndrome, Noonan syndrome with lentiginosis, Bannayan-Riley-Ruvalcaba syndrome
Skin (café-au-lait spots)	McCune-Albright syndrome, Neurofibromatosis type 1, Neurofibromatosis type 2, Watson syndrome
Skin (blue nevi)	Solitary lesions
Thyroid (tumors)	Cowden syndrome, Sporadic thyroid tumors
Testes (Large-cell calcifying Sertoli cell tumor (LCCSCT))	Peutz-Jeghers syndrome
Ovarian (tumors)	Peutz-Jeghers syndrome
Adrenals	Sporadic isolated primary pigmented nodular adrenocortical disease (PPNAD) Isolated micronodular adrenocortical hyperplasia
Adrenocortical tumors	Beckwith-Wiedemann syndrome, Li-Fraumeni syndrome, Multiple Endocrine Neoplasia type 1 (MEN1), Congenital adrenal hyperplasia resulting from 21-hydroxylase deficiency, McCune-Albright syndrome
Pituitary (GH-secreting adenoma)	MEN1, Isolated Familial Somatotropinomas (IFS), Sporadic somatotropinomas
Schwannomas	Neurofibromatosis type 1, Neurofibromatosis type 2, Isolated familial schwannomatosis