NOTE: The Carney Triad is distinct from the Carney Complex of multiple inherited tumors (see below).
Age at diagnosis of the first component: 7 to 48 years of age (82% under 30 years of age)
Classic tumors in the triad:
(1) a stromal or smooth muscle tumor of the stomach
(2) pulmonary chondroma
(3) paraganglioma (originally limited to extra-adrenal sites)
Additional tumors:
(1) nonfunctioning tumors of the adrenal cortex
(2) stromal or smooth muscle tumor in the esophagus, duodenum or jejunum
Types of stromal or smooth muscle tumors (page 549, Carney, 1999):
(1) benign or malignant GI stromal tumor
(2) leiomyoma
(3) leiomyosarcoma (diagnosis in the original series, but GI stromal tumors were not recognized at the time due to the lack of appropriate markers)
Paragangliomas (Table 4, page 546, Carney, 1999):
(1) para-adrenal
(2) pheochromocytoma (intra-adrenal)
(3) sympathetic chain, intra-abdominal or intrathoracic
(4) superior mediastinum
(5) aortopulmonary body
(6) thyroid
(7) head and neck (glomus tympanicum, glomus jugulare, glomus vagale, larynx)
A few patients may have affected siblings (suggesting an inherited trait), but most do not have affected first order relatives.
