Carney complex
Carney complex (not to be confused with the Carney triad) is a rare multiple endocrine neoplasia syndrome, which is autosomal dominant and characterized by 1-4:
-
cardiac myxoma
- often multiple
- seen in two-thirds of patients with Carney complex
- skin pigmentation (blue naevi): especially of the face, trunk, lips, and sclera
Multiple other features are also well recognised including:
- extracardiac myxoma
- breast
- testis
- thyroid
- brain
- adrenal gland: primary pigmented nodular adrenocortical disease (PPNAD)
- pituitary adenoma
- psammomatous melanotic schwannoma
-
testicular tumors
- Sertoli cell tumors: most common
Pathology
Carney complex is due to a mutation of the PRKAR1A gene on the long arm of chromosome 17 (17q) 5.
Mnemonic
One way to remember the differentiation between the Carney triad and the Carney complex is that the Carney Complex has Cardiac findings (myxoma).