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Carney Complex

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 36 Accesses

Synonyms

Carney syndrome; Specific subsets of Carney complex have been termed LAMB (lentigines, atrial myxoma, and blue nevi) and NAME (nevi, atrial myxoma, myxoid neurofibroma, and ephelides) syndromes, respectively.

Definition

First described in 1985, Carney complex is a multisystem tumor syndrome characterized by a variety of nonendocrine and endocrine manifestations including pigmented skin/mucosal lesions, myxomatous tumors of the heart and other tissues, and lesions of adrenal cortex, pituitary gland, thyroid, gonads, and nerve sheath.

Clinical Features

  • Incidence

    Carney complex is rare but is probably underrecognized due to the lack of awareness and overall complexity of different manifestations. Overall, around 750 cases have been documented worldwide since the description of the complex.

  • Age

    The median age at diagnosis is approximately 20 years; however, cases have been diagnosed as early as the second decade of life up to the fifth decade of life.

  • Sex

    Both males and females...

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References and Further Reading

  • Bertherat, J. (2006). Carney complex (CNC). Orphanet Journal of Rare Diseases, 1, 21.

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  • 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, L. S., Bertagna, X., Carney, J. A., & Stratakis, C. A. (2009). Mutations in regulatory subunit type 1A of cyclic adenosine 5′-monophosphate-dependent protein kinase (PRKAR1A): Phenotype analysis in 353 patients and 80 different genotypes. The Journal of Clinical Endocrinology and Metabolism, 94, 2085–2091.

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  • Boikos, S. A., & Stratakis, C. A. (2007). Carney complex: The first 20 years. Current Opinion in Oncology, 19, 24–29.

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  • Carney, J. A., & Young, W. F. (1992). Primary pigmented nodular adrenocorticaldisease and its associated conditions. Endocrinologist, 2, 6–21.

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  • Carney, J. A., Gordon, H., Carpenter, P. C., Shenoy, B. V., & Go, V. L. (1985). The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore), 64, 270–283.

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  • Correa, R., Salpea, P., & Stratakis, C. A. (2015). Carney complex: An update. European Journal of Endocrinology, 173, M85–M97.

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  • Duan, K., & Mete, O. (2017). Hereditary endocrine tumor syndromes: The Clinicaland predictive role of molecular histopathology. AJSP: Reviews & Reports, 22, 246–268.

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  • Hannah-Shmouni, F., Faucz, F. R., & Stratakis, C. A. (2016). Alterations of phosphodiesterases in adrenocortical tumors. Frontiers in Endocrinology (Lausanne), 7, 111.

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  • Kirschner, L. S., Sandrini, F., Monbo, J., Lin, J. P., Carney, J. A., & Stratakis, C. A. (2000). Genetic heterogeneity and spectrum of mutations of the PRKAR1A gene in patients with the carney complex. Human Molecular Genetics, 9, 3037–3046.

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  • Lloyd, R. V., Osamura, R. Y., Kloppel, G., & Rosai, J. (Eds.). (2017). WHO classification of tumours of endocrine organs (4th ed.). IARC: Lyon.

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  • Memon, S. S., Thakkar, K., Patil, V., Jadhav, S., Lila, A. R., Fernandes, G., Bandgar, T. R., & Shah, N. S. (2019). Primary pigmented nodular adrenocortical disease (PPNAD): Single centre experience. Journal of Pediatric Endocrinology & Metabolism, 32(4), 391–397.

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  • Morin, E., Mete, O., Wasserman, J. D., Joshua, A. M., Asa, S. L., & Ezzat, S. (2012). Carney complex with adrenal cortical carcinoma. The Journal of Clinical Endocrinology and Metabolism, 97(2), E202–E206.

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  • Stratakis, C. A., Kirschner, L. S., & Carney, J. A. (1998). Carney complex: Diagnosis and management of the complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas. American Journal of Medical Genetics, 80, 183–185.

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  • Stratakis, C. A., Kirschner, L. S., & Carney, J. A. (2001). Clinical and molecular features of the Carney complex: Diagnostic criteria and recommendations for patient evaluation. The Journal of Clinical Endocrinology and Metabolism, 86, 4041–4046.

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Author information

Authors and Affiliations

  1. Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada

    Anjelica Hodgson, Sara Pakbaz & Ozgur Mete

  2. Department of Pathology, University Health Network, Toronto, ON, Canada

    Anjelica Hodgson, Sara Pakbaz & Ozgur Mete

  3. Endocrine Oncology Site, The Princess Margaret Cancer Centre, Toronto, ON, Canada

    Ozgur Mete

Authors
  1. Anjelica Hodgson
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  2. Sara Pakbaz
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  3. Ozgur Mete
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Corresponding author

Correspondence to Ozgur Mete .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Hodgson, A., Pakbaz, S., Mete, O. (2020). Carney Complex. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5567-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5567-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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