Carney's triad is a rare nonhereditary condition affecting young females. Its prevalence is less than one in a million population.1 It is characterized by gastrointestinal stromal tumors (GISTs), pulmonary chondroma, and extra-adrenal paraganglioma. The genetic alterations in this triad are yet to be understood. It is more appropriately classified as a novel multiple endocrine neoplasia. We report an interesting case of a young lady incidentally detected with tumors composing the triad.
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):543–552. PMID: 10377927. DOI: 10.4065/74.6.543.
Boikos SA, Xekouki P, Fumagalli E, et al. Carney triad can be (rarely) associated with germline succinate dehydrogenase defects. Eur J Hum Genet 2016;24(4):569–573. DOI: 10.1038/ejhg.2015.142. PMID: 26173966.
Scopsi L, Collini P, Muscolino G. A new observation of the Carney's triad with long follow up period and additional tumors. Cancer Detect Prev 1999;23(5):435–443. DOI: 10.1046/j.1525-1500.1999.99047.x.
Tirumani SH, Tirumani H, Jagannathan JP, et al. MDCT features of succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours. Br J Radiol 2014;87(1043):20140476. PMID: 4207163. DOI: 10.1259/bjr.20140476.
Carney JA, Stratakis CA, Young WF Jr. Adrenal cortical adenoma: the fourth component of the Carney triad and an association with subclinical Cushing syndrome. Am J Surg Pathol 2013;37(8):1140–1149. PMID: 23681078. DOI: 10.1097/PAS.0b013e318285f6a2.
Evans RA, Salisbury JR, Gimson A, et al. Case report: Indolent gastric epithelioidleiomyosarcoma in Carney's triad. Clin Radiol 1990;42:437–439. DOI: 10.1016/S0009-9260(05)80902-X.
McGahan JP. Carney syndrome: usefulness of computed tomography in demonstrating pulmonary chondromas. J Comput Assist Tomogr. 1983 Feb;7(1):137–9. PMID: 6826834.
Carney JA. Carney triad: a syndrome featuring paraganglionic, adrenocortical, and possibly other endocrine tumors. J Clin Endocrinol Metab 2009;94(10):3656–3662. DOI: 10.1210/jc.2009-1156.
Margulies KB, Sheps SG. Carney's triad: guidelines for management. Mayo Clin Proc 1988;63(5):496–502. DOI: 10.1016/s0025-6196(12)65648-1.