[Carney's triad. Apropos of a case of recurrent gastric leiomyosarcoma and bilateral pulmonary chondroma].
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.
Pignatelli
V
V
U.O. di Radiodiagnostica dell'USSL, Università, Pisa.
Campani
D
D
Grassi
L
L
Pingitore
R
R
D002648:Child; D002812:Chondroma / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008175:Lung Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D010236:Paraganglioma, Extra-Adrenal / Q000150:complications*; D011859:Radiography; D013274:Stomach Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D013577:Syndrome
La triade di Carney. A proposito di un caso con leiomiosarcoma gastrico recidivante e condroma polmonare bilaterale.