Carney complex

Last revised by Henry Knipe on 18 Jan 2024

Carney complex (not to be confused with the Carney triad) is a rare multiple endocrine neoplasia syndrome characterized by 1-4:

  • cardiac myxoma

    • often multiple

    • seen in two-thirds of patients with Carney complex

  • skin pigmentation (blue nevi): especially of the face, trunk, lips, and sclera  

Multiple other features are also well recognized including:

Collectively there have been more than 750 cases of Carney complex reported worldwide however the exact prevalence is unknown 6,7. One study of 353 patients found a female predilection (63%) 8.

Carney complex has autosomal dominant inheritance with almost 100% penetrance, related to inactivating mutations or large deletions of the PRKAR1A tumor suppressor gene on chromosome 17q22-24 5,6

One way to remember the differentiation between the Carney triad and the Carney complex is that the Carney Complex has Cardiac findings (myxoma).

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