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Carney Complex

Abstract

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NORD is very grateful to J. Aidan Carney MD, PhD, FRCP, Emeritus Professor of Pathology, Mayo Clinic, for assistance in the preparation of this report.

Synonyms of Carney Complex

  • Carney syndrome
  • CNC
  • familial myxoma
  • lentigines, atrial myxoma, and blue nevi (LAMB) syndrome
  • nevi, atrial myxoma, myxoid neurofibromas, and ephelides (NAME) syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Carney complex is a rare genetic disorder characterized by multiple benign tumors (multiple neoplasia) most often affecting the heart, skin and endocrine system and abnormalities in skin coloring (pigment) resulting in a spotty appearance to the skin of affected areas. Benign tumors of connective tissue (myxomas) are common in individuals with Carney complex and, most often, are found in the heart where they can potentially cause serious, life-threatening complications including stroke, valvular obstruction or heart failure. A wide variety of endocrine abnormalities potentially can occur in Carney complex affecting a variety of glands. Additional tumors include myxomas affecting the skin and nerve sheath tumors (schwannomas). Skin pigment abnormalities include tiny flat (freckle-like) black or brown spots (multiple lentigines) and small, blue or bluish-black spots (blue nevi). The specific symptoms and severity of Carney complex can vary greatly from one person to another. In many cases, Carney complex is due to mutations of the PRKAR1A gene. The mutation can occur randomly for no apparent reason (i.e., new mutation) or be inherited as an autosomal dominant trait.

Carney complex is a different disorder from Carney triad. Carney triad encompasses three types of tumors: a gastric stromal sarcoma; functioning extra-adrenal paragangliomas; and pulmonary chondromas. Although these two disorders are completely unrelated, both have sometimes been referred to as Carney syndrome, causing confusion. This report deals solely with Carney complex.

Carney Complex Resources

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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