Carney triad

Carney triad is a rare syndrome defined by the coexistence of three tumors:

• extra-adrenal paraganglioma
  • 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.

Terminology

It is not to be confused with the related Carney-Stratakis syndrome, or the unrelated Carney complex.

• 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.