Carney's triad: guidelines for management

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.