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).