Literature DB >> 2850598

[Carney's triad. Apropos of a case of recurrent gastric leiomyosarcoma and bilateral pulmonary chondroma].

V Pignatelli1, D Campani, L Grassi, R Pingitore.   

Abstract

Carney's triad is a rare syndrome characterized by gastric leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Twenty-seven cases are reported in literature: the most commonly observed neoplasm is gastric leiomyosarcoma (26/27) followed by lung tumor (22/27), and gastric paraganglioma (15/27). Three patients bearing a gastric leiomyosarcoma developed liver metastases, and 6 had a local recurrence after some years. The occurrence of paraganglioma makes the prognosis worse: 4 deaths from local infiltration, metastases or severe hypertension. The triad only occurred in 9/27 cases, mostly young female patients. A common embryologic origin is difficult to assess. The case of a young female patient with recurrent gastric leiomyosarcoma (12 years later) and bilateral pulmonary chondroma is described. It is important that patients with any of the three tumors be periodically examined.

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Year:  1988        PMID: 2850598

Source DB:  PubMed          Journal:  Radiol Med        ISSN: 0033-8362            Impact factor:   3.469


  1 in total

1.  Adrenal cortical adenoma: the fourth component of the Carney triad and an association with subclinical Cushing syndrome.

Authors:  J Aidan Carney; Constantine A Stratakis; William F Young
Journal:  Am J Surg Pathol       Date:  2013-08       Impact factor: 6.394

  1 in total

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