- 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:
- extracardiac myxoma
- adrenal gland: primary pigmented nodular adrenocortical disease (PPNAD)
- pituitary adenoma
- psammomatous melanotic schwannoma
- testicular tumors
- Sertoli cell tumors: most common
Collectively there have been more than 750 cases of Carney complex reported worldwide however the exact prevalence is unknown . One study of 353 patients found a female predilection (63%) .
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 .
One way to remember the differentiation between the Carney triad and the Carney complex is that the Carney Complex has Cardiac findings (myxoma).