Carney Complex

Carney complex

Carney complex is an autosomal dominant disorder characterised by cardiac myxomas, lentiginosis and endocrine abnormalities. The mutation associated with Carney complex is in the PRKAR1-α gene. This gene is involved in the synthesis of a subunit of protein kinase A. Lentginosis (spotty skin pigmentation) occurs often on the face and oral mucosa. Myxomatous lesions may also occur in the skin and breast.

LAMB (Lentigines, Atrial myxomas, Mucocutaneous myxomas, and Blue nevi) syndrome and NAME (Nevi, Atrial myxoma, Myxoid neurofibromas, and Ephelides) syndrome are the other names sometimes given for this array of findings.

The adrenal disorder associated with Carney complex is called primary pigmented nodular adrenocortical disease. This can lead on to hypercortisolism and Cushing’s syndrome. Other endocrine tumors associated are those of thyroid, testis and ovaries. Pituitary adenomas may also occur.
Psammomatous melanotic schwannoma is another association.