Carney Triad

Carney et al. (1977) reported the association of gastric (epithelioid) leiomyosarcoma, functioning extraadrenal paraganglioma, and pulmonary chondroma in 2 patients and the association of 2 of the 3 tumors in 5 other patients. All were unrelated young women. One patient also had a nonfunctioning adrenocortical adenoma (Margulies and Sheps, 1988). The tumor pattern, namely multifocal lesions in multiple organs in young patients, suggested an inherited disorder, but such was not found. Carney (1983) reviewed findings in 24 affected patients (including 2 males), 6 of whom had the 3 tumors. The gastric neoplasm had metastasized in 8 patients, demonstrating its malignant nature. Some of the paragangliomas were nonfunctioning in the second series. Carney (1999) reviewed the findings in a total of 79 patients (including 48 patients recognized since 1983). Forty-two (53%) had gastric and pulmonary tumors, the most common combination. The longest interval between detection of the first and second components was 26 years (mean, 8.4 years; median, 6 years). Two patients each had a sib with one component of the triad. Both had gastric sarcomas and paragangliomas. Carney (1999) noted that gastric stromal sarcoma had not occurred in 549 patients (290 males and 259 females) from 115 kindreds with familial paragangliomas (168000). Thus the patients mentioned do not represent that disorder.

Appelman (1999) reviewed gastrointestinal stromal tumors (GISTs) in general. Gastric stromal tumors, the largest group of GISTs, occur in several varieties. Some are composed of spindle cells, while in others, the cells are round or epithelioid, and there are benign and malignant variants of both cell types.