It is characterized by the presence of multiple cardiac and extracardiac myxomas, spotty skin pigmentation, schwannomas and endocrine tumors, such as GH-secreting PitNETs, corticotroph tumors, and ACTH-independent Cushing syndrome known as primary pigmented nodular adrenocortical disease (PPNAD), and thyroid and gonadal tumors [51,52]. Here, POMC is linked to Cushing syndrome.