Carney complex: A multiple neoplasia syndrome with cardiac, endocrine, cutaneous, and neural tumors together with spotty pigmentation of the skin, particularly on the face, lips, and trunk, and mucosa. The Carney complex may simultaneously involve multiple endocrine glands such as the pituitary, adrenals, and testes. The cardiac tumors are myxomas which can arise in any chamber of the heart. Although almost always benign, cardiac myxomas are associated with stroke from tumor embolism and with heart failure from obstruction of a valve. Myxomas also may occur in the breast, testis, thyroid, brain, or adrenal gland. Systemic symptoms such as fever, arthralgia, and a lupuslike rashes may accompany the myxomas (and may be due to the production of the proinflammatory cytokine interleukin-6 by the myxoma).
Carney complex is inherited in an autosomal dominant manner. There are two genetically distinct forms. Type I Carney complex is due to mutation of the PRKAR1A gene encoding the protein kinase A regulatory subunit 1-alpha on chromosome 17q23-q24. Type II Carney complex is linked to chromosome 2.
Named after J. Alden Carney who with colleagues described "the complex of myxomas, spotty pigmentation, and endocrine overactivity" in 1985. The Carney complex includes the LAMB syndrome (lentigines, atrial myxomas, mucocutaneous myxomas, and blue nevi) and the NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma, and ephelides).
Primary tumors of the heart are rare in all age groups.
Carney complex is a disorder characterized by an increased risk of several types of tumors. Affected individuals also usually have changes in skin coloring ...
Carney complex is a hereditary condition associated with spotty skin pigmentation, myxomas (benign [noncancerous] connective tissue tumors), and benign or cancerous ...
Disease characteristics. Carney complex (CNC) is characterized by skin pigmentary abnormalities, myxomas, endocrine tumors or overactivity, and schwannomas.
Carney complex Monika Srivastava MD Dermatology Online Journal 10 (3): 11 From the Ronald O. Perelman Department of Dermatology, New York University