Case 22 - Carney Triad (Pentad) and Adrenal Adenoma With Clinically Important Cortisol Secretory Autonomy

Abstract

Carney triad (described in 1977) is a rare, nonfamilial, multitumoral syndrome, with three tumors in the initial description: gastrointestinal stromal tumor (GIST), pulmonary chondroma, and extraadrenal paraganglioma. Subsequently, two other tumors, adrenal cortical adenoma and esophageal leiomyoma, were added as components—thus Carney triad is actually a “pentad.” Although it is rare, it is important for endocrinologists to be aware of this disorder because of the links to paraganglioma and adrenocortical tumors. The adrenocortical tumors are usually nonfunctioning adenomas. However, as highlighted in the case described herein, the adrenocortical tumor can secrete cortisol autonomously and lead to a perioperative catastrophe if not recognized before surgery.