Carney's triad with recurrent gastric leiomyoblastoma

The triad of gastric leiomyoblastoma, extra-adrenal paraganglioma, and pulmonary chondroma was first described by Carney in 1977. Fewer than 30 patients with this syndrome have since been reported. In most patients the initial lesion has been a gastric leiomyoblastoma. The patient we describe had recurrences of a gastric leiomyoblastoma 15 and 17 years, respectively, after the initial gastric resection. Subsequently, multiple pulmonary lesions were excised and confirmed to be chondromas, but no evidence of a paraganglioma has yet been identified. Gastric leiomyoblastomas in patients with Carney's triad frequently recur; however, the prognosis is quite favorable in contrast to that of sporadic cases. Whenever a patient with one of the classical lesions of Carney's triad is encountered, the possibility of this unusual syndrome should be considered. The evaluation and follow-up of these patients should include a search for the other two components.