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First published on April 2, 2008
Angiology 2008, doi:10.1177/0003319707305981
© 2008 SAGE Publications

Article

Management of Primary Pulmonary Artery Sarcoma: Experience of a Single Center

Covadonga Fernández-Golfin, MD, Pilar Escribano, MD, PhD*, José Cortina, MD, Rocío Tello, MD, PhD, Felipe Hernández, MD, Fernando López-Rios, MD, Juan Delgado Jiménez, MD, and Carlos Saenz de la Calzada, MD, PhD

Hospital Universitario "12 de octubre"

* To whom correspondence should be addressed. E-mail: pilar.escribano{at}telefonica.net.


  Abstract
Primary sarcoma of the pulmonary artery (PSPA) is extremely rare. Many cases are misdiagnosed as pulmonary arterial hypertension (PAH) because of chronic thromboembolic disease (CTD). Four cases of PSPA with the initial misdiagnosis are reported. The presence of a unique mass in the main pulmonary artery or proximal branches, rapidly progressive dyspnea, and constitutional symptoms should raise the suspicion of PSPA. The pathological diagnosis is usually confirmed during surgery, which is done along with adjuvant chemotherapy, the treatment of choice.


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