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

Covadonga Fernández-Golfín, MD

Department of Cardiology, Hospital Virgen de la Salud, Toledo

Pilar Escribano, MD, PhD

Department of Cardiology, Hospital Virgen de la Salud, Toledo, Departments of Cardiac Surgery, Hospital Universitario "12 de octubre," Madrid, Spain, pilar.escribano{at}telefonica.net

José Cortina, MD

Departments of Cardiac Surgery, Hospital Universitario "12 de octubre," Madrid, Spain

Rocío Tello, MD, PhD

Departments of Cardiac Surgery, Hospital Universitario "12 de octubre," Madrid, Spain

Felipe Hernández, MD

Departments of Cardiac Surgery, Hospital Universitario "12 de octubre," Madrid, Spain

Fernando López-Rios, MD

Pathology, Hospital Universitario "12 de octubre," Madrid, Spain

Juan Delgado Jiménez, MD

Pathology, Hospital Universitario "12 de octubre," Madrid, Spain

Carlos Saenz de la Calzada, MD, PhD

Pathology (FLR, JDJ, CSC), Hospital Universitario "12 de octubre," Madrid, Spain

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.

Key Words: primary sarcoma of the pulmonary artery • pulmonary hypertension • pulmonary thromboembolic disease • diagnosis • treatment

This version was published on October 1, 2008

Angiology, Vol. 59, No. 5, 636-639 (2008)
DOI: 10.1177/0003319707305981
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