Carney Complex

Synonyms

LAMB; lentigineses, atrial myxoma, mucocutaneous myxoma, blue nevi; NAME; nevi, atrial myxoma, myxoid neurofibroma, ephelide; CNC

Definition

The Carney complex (CNC) is a dominant autosomal hereditary multiple neoplasia syndrome characterized mainly by cardiac myxomas, spotty skin pigmentation, and endocrine tumors. It was first described in 1985 by J. Aidan Carney, a pathologist at the Mayo Clinic.

Characteristics

The manifestations of CNC can be numerous and vary between patients. Even in the same kindred, phenotypic variability can be observed. The estimated frequencies of these manifestations are listed in Table 1. Endocrine, dermatologic, and cardiac anomalies are the main manifestations of the disease. The lentiginosis is observed in most patients and is so characteristic that can makes the diagnosis. It appears as small brown to black macules typically located around the upper and lower lips, on the eyelids, ears, and the genital area. Multiple