complex is a multiple endocrine neoplasia (M EN ) syndrome that aþ ects the adrenal u Carney cortex, the pituitary and thyroid g lands, and the gonads. T he complex is also associated with skin and mucosa pigmentation abnormalities and myxoid and other neoplasms of mesenchymal and neural crest orig in. T hus, this syndrome also belongs to another group of genetic disorders, the lentiginoses (or lentigenoses), which include the Peutz-J eghers, LEOPARD, arterial
dissections and lentiginosis, and Laugier-Hunziker syndromes, Cowden disease and Ruvalcaba-M yhre-Smith (Bannayan-Zonana) syndrome and the centrofacial, benign patterned and segmental lentiginoses, all of which can be associated with a variety of developmental defects. T he inheritance of Carney complex, just like that of the other M EN s and the lentiginoses, is autosomal dominant. Genetic loci or g enes have been identiüed f or Carney complex, Peutz-J eghers and Ruvalcaba-M yhre-Smith syndromes, but not for other lentiginoses.Elucidation of the molecular defects responsible for these disorders is expected to shed light on aspects of early neural crest diþ erentiation, the regulation of pigmentation, the development of autonomous endocrine f unction, and endocrine and nonendocrine tumorigenesis.