Carney's triad--gastric leiomyosarcoma, pulmonary chondroma, and extra-adrenal 
paraganglioma--is a syndrome that occurs primarily in young women. To date, 28 
patients with at least two of these individually unusual or rare neoplasms have 
been described. This updated case report of one of the originally described 
patients with Carney's triad highlights several clinically important features of 
this unusual syndrome: (1) the multicentricity of both the paragangliomas and 
the epithelioid leiomyosarcomas, (2) the often indolent progression of 
metastatic leiomyosarcoma, (3) the potential for late recurrences, and (4) the 
importance of distinguishing intra-adrenal from periadrenal 
catecholamine-producing tumors (paragangliomas). Localization of paragangliomas 
is facilitated by two relatively new techniques--131I-metaiodobenzylguanidine 
scanning (a scintigraphic technique with high specificity for 
catecholamine-producing tumors) and two-dimensional echocardiography (which can 
noninvasively localize and demonstrate the anatomic relationships of 
aorticopulmonary paragangliomas). In patients with this syndrome, new or 
recurrent tumors frequently manifest after unusually long asymptomatic 
intervals. We outline an approach for continued follow-up of patients with one 
or more of the three neoplasms that constitute the syndrome. Rigorous long-term 
screening of these patients should not only lead to early recognition and 
resection of recurrent or new tumors but also enhance our understanding of this 
intriguing syndrome.