Carney triad

Abstract

Carney triad is a rare multitumoral syndrome of unknown etiology that was first described in 1977. The neoplasms affect the stomach, lungs, paraganglionic system, adrenal cortex, and esophagus. Approximately 150 cases have been identified. The disorder occurs in young women primarily and is not familial. Multifocal tumors develop in the organs affected. No patient has had tumors in all 5 organs. One patient had tumor in 4 organs; most patients have them in 2. Thus, the syndrome is usually only partially expressed. The gastric tumors are malignant and metastasize to the liver, peritoneum, and lymph nodes. The lung, adrenal, and esophageal tumors are benign. The paraganglionic tumors are usually benign. Long-term follow-up shows that the syndrome is a chronic, persistent, and generally indolent condition whose outcome is largely dependent on the behavior of the metastases of the gastric sarcoma. Among the 79 affected patients described in 1999 (average follow-up 8 years, 64 were alive, 19 were apparently free of tumors, 45 had residual or metastatic disease, and 15 were dead (10 died of causes related to the syndrome).