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seen in two-thirds of patients with Carney complex
skin pigmentation (blue nevi): especially of the face, trunk, lips, and sclera
Multiple other features are also well recognized including:
adrenal gland: primary pigmented nodular adrenocortical disease (PPNAD)
Sertoli cell tumors: most common
Collectively there have been more than 750 cases of Carney complex reported worldwide however the exact prevalence is unknown 6,7. One study of 353 patients found a female predilection (63%) 8.
Carney complex has autosomal dominant inheritance with almost 100% penetrance, related to inactivating mutations or large deletions of the PRKAR1A tumor suppressor gene on chromosome 17q22-24 5,6.
One way to remember the differentiation between the Carney triad and the Carney complex is that the Carney Complex has Cardiac findings (myxoma).
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- 8. Bertherat J, Horvath A, Groussin L, Grabar S, Boikos S, Cazabat L, Libe R, René-Corail F, Stergiopoulos S, Bourdeau I, Bei T, Clauser E, Calender A, Kirschner LS, Bertagna X, Carney JA, Stratakis CA. Mutations in regulatory subunit type 1A of cyclic adenosine 5'-monophosphate-dependent protein kinase (PRKAR1A): phenotype analysis in 353 patients and 80 different genotypes. (2009) The Journal of clinical endocrinology and metabolism. 94 (6): 2085-91. doi:10.1210/jc.2008-2333 - Pubmed