Citation, DOI, disclosures and article data
Citation:
Gaillard F, Knipe H, Deng F, et al. Carney complex. Reference article, Radiopaedia.org (Accessed on 26 Apr 2024) https://doi.org/10.53347/rID-1053
Disclosures:
At the time the article was last revised Henry Knipe had the following disclosures:
- Integral Diagnostics, Shareholder (ongoing)
- Micro-X Ltd, Shareholder (ongoing)
These were assessed during peer review and were determined to
not be relevant to the changes that were made.
View Henry Knipe's current disclosures
Carney complex (not to be confused with the Carney triad) is a rare multiple endocrine neoplasia syndrome characterized by 1-4:
Multiple other features are also well recognized including:
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
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3. Carney Complex by Craig T Basson from emedicine.com. Carney Complex
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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
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5. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
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6. Correa R, Correa SP, Correa SC, Correa. Carney complex: an update. (2015) European journal of endocrinology. doi:10.1530/EJE-15-0209 - Pubmed
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7. Espiard S, Espiard BJ, Espiard. Carney complex. (2013) Frontiers of hormone research. doi:10.1159/000345669 - Pubmed
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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
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