- Edward W Cowen, MD, MHSc
Edward W Cowen, MD, MHSc
- Director, American Board of Dermatology
- Newton, MA, USA;
- Fellow, American Academy of Dermatology
- Schaumburg, IL, USA;
- Senior Clinician and Head, Dermatology Consultation Service
- Center for Cancer Research, National Cancer Institute,
- National Institutes of Health
- Bethesda, MD, USA
Carney complex (CNC, MIM #160980) is a rare multiple endocrine neoplasia syndrome characterized by distinctive pigmented lesions of the skin and mucosal surfaces, cardiac and noncardiac myxomatous tumors, and multiple endocrine tumors . CNC is most frequently associated with mutations in the protein kinase A type I-alpha regulatory subunit gene (PRKAR1A) and is inherited in an autosomal dominant fashion. Approximately 25 percent of cases occur sporadically, as a result of a de novo mutation.
CNC has been previously called NAME (nevi, atrial myxoma, ephelides) and LAMB (lentigines, atrial myxoma, blue nevi) syndrome. It should not be confused with the Carney triad, which consists of paragangliomas, gastric stromal tumors, and pulmonary chondromas . (See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology" and "Epidemiology, classification, clinical presentation, prognostic features, and diagnostic work-up of gastrointestinal mesenchymal neoplasms including GIST".)
This topic review will focus on the cutaneous manifestations, diagnosis, and differential diagnosis of CNC. Endocrine and nonendocrine tumors associated with CNC are discussed separately.
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