Citation, DOI & article data
Carney triad is a rare syndrome defined by the coexistence of three tumors:
- initially, only functioning extra-adrenal paragangliomas were included, but subsequent work includes non-functioning extra-adrenal paragangliomas 1
- gastric gastrointestinal stromal tumors (GIST)
- pulmonary chondroma
In most cases, only 2 of the 3 tumors are present at the time of diagnosis, with the most common combination being GIST and pulmonary chondromas. Although not considered part of the triad, there is an increased incidence of adrenocortical adenoma. It typically affects young people. No underlying genetic mutation has been identified.
- Memory tip : It may be helpful to associate "T" in GIST with Carney Triad ; "X" in MyXoma can be associated with Carney CompleX.
History and etymology
First described by J Aidan Carney, an American professor of pathology, and colleagues in 1977 5.
- 1. Carney JA. Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin. Proc. 1999;74 (6): 543-52. doi:10.4065/74.6.543 - Pubmed citation
- 2. Carney JA, Sheps SG, Go VL et-al. The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. N. Engl. J. Med. 1977;296 (26): 1517-8. doi:10.1056/NEJM197706302962609 - Pubmed citation
- 3. Chew F, Banks K. American Journal of Roentgenology. 2006;187 (3_supplement): . doi:10.2214/AJR.06.0521
- 4. Alrashdi I, Bano G, Maher ER, Hodgson SV. Carney triad versus Carney Stratakis syndrome: two cases which illustrate the difficulty in distinguishing between these conditions in individual patients. Familial cancer. 9 (3): 443-7. doi:10.1007/s10689-010-9323-z - Pubmed
- 5. Aidan Carney, J. The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer. 1979;43 (1): 374–382. doi: 10.1002/1097-0142(197901)43:1<374::AID-CNCR2820430152>3.0.CO;2-T