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Carney complex

Carney complex is an autosomal dominant syndrome charachterized by superficial angiomyxomas, cardiac myxomas, pigmented skin lesions (lentigines), and endocrine hyperactivity. The condition does not show any racial predilection. Males and females are equally affected. Transmission from mother is more common due to the fact that male patients develop testicular tumors impairing fertility. Both familial and sporadic cases are reported. The majority of Carney complex cases is caused by a mutation of PRKAR1α gene on chromosome 17q22-24, which codes for the protein kinase A (PKA) type 1α regulatory subunit.

Endocrine manifestations of the syndrome result from hormone-producing pituitary adenomas (acromegaly, sexual precocity, hyperprolactinemia), and primary pigmented nodular adrenal disease, or PPNAD (Cushing syndrome). Other common manifestations include psammomatous melanotic schwannomas, myxoid fibroadenomas of breast, large-cell calcifying Sertoli cell tumors (LCCSCT) and Leydig cell tumors of the testes, and follicular adenomas of the thyroid.

Carney complex is also known by the acronymic names of LAMB syndrome (Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi) and NAME syndrome (Nevi, Atrial myxomas, Myxoid neurofibromas, and Ephelides).

See also: Carney triad

Last updated: May 9, 2012, 11:14 am EST

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Carney complex

Carney complex
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Angiomyxomas are one of the components of Carney complex