Carney complex

Definition

• Rare multiple neoplasia syndrome associated with distinctive cutaneous lesions
• Inactivating mutations in the protein kinase A type I-alpha regulatory subunit (PRKAR1A) gene
• AD
• Previously called NAME (nevi, atrial myxoma, ephelides) and LAMB (lentigines, atrial myxoma, blue nеvi) syndrome.

Clinical

Cutaneous

• Lentiginous skin pigmentation - 70 to 80 percent of patients. O
  • Often have a periorificial distribution and may be found on the lid margin and lacrimal caruncle.
• Dark “ink spot” lentigines
• Multiple blue nеvi
• Cutaneous myxomas.
  • Benign dermal tumors presenting as sharply demarcated subcutaneous nodules, a few millimeters to 1.5 cm in diameter

Extracutaneous

• Primary pigmented nodular adrenocortical disease (ΡPΝAD)
• Testicular large cell calcifying Sertoli tumors
• Pituitary adenomas
• Thyroid adenomas and carcinomas
• Ovarian cysts.
• Cardiac myxomas
• psammomatous melanotic schwannomas
• Breast ductal adenomas
• Osteochondromyxomas