CARNEY COMPLEX

By Gary M. White, MD

A large Blue Nevus in the scalp of a patient with Carney Complex A large blue nevus in the scalp in a patient with the Carney Complex. Courtesy Theodore Sebastian, MD


In the Carney Complex (CC), also called LAMB syndrome, multiple pigmented macules (lentigines and blue nevi) occur in association with myxomas (of the skin, breasts, and heart), functioning endocrine tumors (e.g., bilateral primary pigmented nodular adrenocortical hyperplasia, growth hormone-secreting pituitary adenomas), psammomatous melanotic schwannoma, and testicular tumors.

Clinical

Multiple pigmented macules may occur on the face (especially about the eyes and ears), groin (both penis and vulva), or elsewhere. These lentigenes may be present at birth or appear during childhood. They tend to increase in number around puberty and often fade in middle age.

Blue nevi are more common in CC.

Myxomas may occur on the skin as papules, nodules, and plaques anywhere on the skin (e.g. ear, nipple).

References

Cutaneous Myxomas. Washington University Dermatology Case of the Month February-March

Fam Cancer. Sep 2011; 10(3): 481–490

Insights Imaging. 2013 February;4(1):119-133
Myxoma on the ear lobe. J Cutan Pathol. Jan 2012; 39(1): 68–71.

19-year-old woman with myxomas on the nipples. Korean J Pathol. 2012 December;46(6):595-600

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