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March 13, 1995

Gastric Stump Carcinoma Occurring in a Patient With Carney's Triad 38 Years After Partial Gastrectomy for Epithelioid Leiomyosarcoma

Author Affiliations

New York, NY

Arch Intern Med. 1995;155(5):544-547. doi:10.1001/archinte.1995.00430050124017

Carney's triad is a rare syndrome of unusual tumors first described in 1977 by Carney et al.1 Articles in which over 40 patients have been described have appeared in the literature since then, with a review of 36 cases summarized in 1993.2 The triad includes gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma. The tumors are commonly multicentric and only one third of the patients who were affected have manifested all three, most exhibiting two of the three tumors.2 The triad affects predominantly young women, is usually heralded by signs and symptoms related to the gastric tumor, and patients can live years to decades with metastases of the gastric leiomyosarcoma. The cause of this rare syndrome has yet to be studied.

We provide follow-up on a previously described patient with Carney's triad3 who since developed signet-ring cell carcinoma of the gastric stump. The patient was