- often multiple
- 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:
- extracardiac myxoma
- adrenal gland: primary pigmented nodular adrenocortical disease (PPNAD)
- pituitary adenoma
- psammomatous melanotic schwannoma
- 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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- 2. Schoepf UJ. CT of the heart, principles and applications. Springer. (2005) ISBN:1592598188. Read it at Google Books - Find it at Amazon
- 3. Carney Complex by Craig T Basson from emedicine.com. Carney Complex
- 4. Grebenc ML, Rosado de christenson ML, Burke AP et-al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 20 (4): 1073-103. Radiographics (full text) - Pubmed citation
- 5. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
- 6. Correa R, Correa SP, Correa SC, Correa. Carney complex: an update. (2015) European journal of endocrinology. doi:10.1530/EJE-15-0209 - Pubmed
- 7. Espiard S, Espiard BJ, Espiard. Carney complex. (2013) Frontiers of hormone research. doi:10.1159/000345669 - Pubmed
- 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