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Carney Triad

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

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

  • 47 Accesses

Definition

Originally described in 1977, the Carney triad is a tumor syndrome classically consisting of three tumors: paraganglioma, gastrointestinal stromal tumor (GIST), and pulmonary chondroma. GISTs are the most common presenting tumors followed by pulmonary chondromas and lastly, paragangliomas. Approximately 20% of affected patients present with the classic three tumor types; however, the majority of affected patients present with two of the three tumors, usually GIST and pulmonary chondroma. The interval between the development of the sentinel tumor and a subsequent metachronous tumor has been reported to be as long as two decades or more. Nonfunctioning benign adrenocortical tumors have been described as a possible fourth element of the syndrome, in addition to esophageal leiomyomas and pheochromocytomas (intra-adrenal sympathetic paragangliomas).

Clinical Features

  • Incidence

    Carney triad is very rare with less than 100 reported cases.

  • Age

    Most affected patients are diagnosed in...

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References and Further Reading

  • Boikos, S. A., Xekouki, P., Fumagalli, E., Faucz, F. R., Raygada, M., Szarek, E., Ball, E., Kim, S. Y., Miettinen, M., Helman, L. J., Carney, J. A., Pacak, K., & Stratakis, C. A. (2016). Carney triad can be (rarely) associated with germline succinate dehydrogenase defects. European Journal of Human Genetics, 24, 569–573.

    Article  CAS  Google Scholar 

  • Carney, J. A. (2009). Carney triad: A syndrome featuring paraganglionic, adrenocortical, and possibly other endocrine tumors. The Journal of Clinical Endocrinology and Metabolism, 94(10), 3656–3662.

    Article  CAS  Google Scholar 

  • Carney, J. A., Sheps, S. G., Go, V. L., & Gordon, H. (1977). The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. The New England Journal of Medicine, 296, 1517–1518.

    Article  CAS  Google Scholar 

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

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  • Gaal, J., Stratakis, C. A., Carney, J. A., Ball, E. R., Korpershoek, E., Lodish, M. B., Levy, I., Xekouki, P., van Nederveen, F. H., den Bakker, M. A., O’Sullivan, M., Dinjens, W. N., & de Krijger, R. R. (2011). SDHB immunohistochemistry: A useful tool in the diagnosis of Carney-Stratakis and Carney triad gastrointestinal stromal tumors. Modern Pathology, 24, 147–151.

    Article  CAS  Google Scholar 

  • Matyakhina, L., Bei, T. A., McWhinney, S. R., Pasini, B., Cameron, S., Gunawan, B., Stergiopoulos, S. G., Boikos, S., Muchow, M., Dutra, A., Pak, E., Campo, E., Cid, M. C., Gomez, F., Gaillard, R. C., Assie, G., Füzesi, L., Baysal, B. E., Eng, C., Carney, J. A., & Stratakis, C. A. (2007). Genetics of carney triad: Recurrent losses at chromosome 1 but lack of germline mutations in genes associated with paragangliomas and gastrointestinal stromal tumors. The Journal of Clinical Endocrinology and Metabolism, 92, 2938–2943.

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  • Stratakis, C. A., & Carney, J. A. (2009). The triad of paragangliomas, gastric stromal tumors and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): Molecular genetics and clinical implications. Journal of Internal Medicine, 266(1), 43.

    Article  CAS  Google Scholar 

  • Zhang, L., Smyrk, T. C., Young, W. F., Jr., Stratakis, C. A., & Carney, J. A. (2010). Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: Findings in 104 cases. The American Journal of Surgical Pathology, 34, 53–64.

    Article  Google Scholar 

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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 Triad. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5215-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5215-1

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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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