Definition of Carney complex

Reviewed on 6/3/2021

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, joint pains, and a lupus-like butterfly facial 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).


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