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Objective: To investigate the natural history of the triad of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma, a rare syndrome of unknown cause primarily affecting young women., Methods: Mayo Clinic records, the world literature, and the author's files were searched for patients with all or 2 of the 3 tumors., Results: Seventy-nine patients, 67 women and 12 men, were identified, 17 (22%) with the 3 tumors and 62 (78%) with 2 tumors. 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). Follow-up ranged from 1 year to 49 years (mean, 20.6 years; median, 20 years). Sixty-four patients (81%) were alive, 19 (24%) apparently free of disease and 45 (57%) with residual or metastatic tumors. Thirty-two patients (41%) had had 1 or more local recurrences of the gastric sarcoma; the longest interval to first recurrence was 36 years. Twenty-one survivors (27%) had hepatic metastatic gastric sarcoma with follow-up of 1 year to 25 years (mean, 9.3 years; median, 7 years). Fifteen patients (19%) were dead, 10 (13%) of whom died of the disorder. Ten patients (13%) had nonfunctioning adrenocortical tumors. Two patients each had a sibling with 1 component of the triad., Conclusions: The triad is a chronic, persistent, and indolent disease. Benign adrenocortical tumors are a component of the condition. The disorder may be familial.

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.