Pulmonary Arterial Hypertension in a Patient with Cowden Syndrome and the PTEN Mutation. (1st December 2014)

• Record Type: Journal Article
• Authors: Ha, Duc; Mester, Jessica; Eng, Charis; Farha, Samar
• Is Part Of: Pulmonary circulation. Volume 4:Number 4(2014)
• Page Start: 728
• Page End: 731
• Publication Date: 2014-12-01
• Subjects: pulmonary hypertension -- PTEN mutation -- Cowden syndrome; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1086/678552
• Languages: English
• ISSNs: 2045-8932

Abstract

Abstract : The pathogenesis of pulmonary arterial hypertension (PAH) exhibits many neoplastic‐like features. Cowden syndrome is a difficult‐to‐recognize heritable cancer syndrome caused by a germline mutation in the phosphatase‐and‐tensin homolog deleted on the chromosome 10 ( PTEN ) gene. PTEN regulation has been implicated in cancer development and, more recently, PAH pathogenesis. Here we report a case of PAH in a patient with Cowden syndrome and the response to pulmonary vasodilators.