Preferred Label : Carney complex;
ICD-11 definition : Carney complex is a dominantly inherited hamartoneoplastic syndrome caused bs caused
by mutation in the protein kinase A regulatory subunit-1-alpha gene on chromosome
17q. It is characterized by spotty skin and mucosal pigmentation (lentiginosis and
blue naevi); overactivity affecting multiple endocrine glands, the manifestations
of which may include acromegaly, Cushing syndrome and tumours of the thyroid and testes;
and myxomas in the heart, skin and breast.;
ICD-11 synonym : Myxoma - spotty pigmentation - endocrine overactivity; Carney syndrome;
ICD-11 inclusion : LAMB - [lentigines, atrial myxomas, mucocutaneous myxomas, and blue naevi] syndrome;
Origin ID : 1051158630;
Currated CISMeF NLP mapping
Carney complex is a dominantly inherited hamartoneoplastic syndrome caused bs caused
by mutation in the protein kinase A regulatory subunit-1-alpha gene on chromosome
17q. It is characterized by spotty skin and mucosal pigmentation (lentiginosis and
blue naevi); overactivity affecting multiple endocrine glands, the manifestations
of which may include acromegaly, Cushing syndrome and tumours of the thyroid and testes;
and myxomas in the heart, skin and breast.
