| Literature DB >> 2850598 |
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.Entities:
Mesh:
Year: 1988 PMID: 2850598
Source DB: PubMed Journal: Radiol Med ISSN: 0033-8362 Impact factor: 3.469