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

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.

Keywords

primary sarcoma of the pulmonary artery pulmonary hypertension pulmonary thromboembolic disease diagnosis treatment

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References

Cox JE , Chiles C. , Aquino S. , Savage P. , Oaks T. Pulmonary artery sarcomas: a review of clinical and radiologic features. J Comput Assist Tomogr. 1997;21:750-755.

Krüger I. , Borowski A. , Horst M. , de Vivie ER , Thiessen P. , Gross-Fengels W. Symptoms, diagnosis and therapy of primary sarcomas of the pulmonary artery . Thorac Cardiovasc Surg. 1989;38:91-95.

Burke A. , Virmani R. Sarcomas of the great vessels: a clinicopathologic study. Cancer. 1993;71:1761-1773.

Eunhee S. Tumors of the pulmonary vasculature. Cardiol Clin . 2004;22:431-440.

Parish J. , Rosenow E. , Swensen S. , Crotty T. Pulmonary artery sarcoma: clinical features. Chest . 1996;110:1480-1488.

Anderson M. , Kriett J. , Kapelanski D. , Tarazi R. , Jamieson SW Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg. 1995;59:1487-1490.

Delany S. , Doyle T. , Bunton R. , Hung NA , Joblin LU , Taylor DR Pulmonary artery sarcoma mimicking pulmonary embolism. Chest . 1993;103:1631-1633.

Choong C. , Lawton J. , Moon M. , Damiano R. Failure of medical therapy for pulmonary "thromboembolic" disease: beware the unsuspected primary sarcoma of the pulmonary artery. J Thorac Cardiovasc Surg. 2004;128: 763-765.

Yi CA , Lee KS , Choe YH , Han D. , Kwon OJ , Kim S. Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease. J Comput Assist Tomogr. 2004;28:34-39.

10.

Yamada N. , Kamei S. , Yasuda F. , Isaka N. , Yada I. , Nakano T. Primary leiomyosarcoma of the pulmonary artery confirmed by catheter suction biopsy. Chest. 1998;113:555-556.

11.

Vaporciyan A. , Rice D. , Correa A. , et al. Resection of advanced thoracic malignancies requiring cardiopulmonary bypass. Eur J Cardiothorac Surg . 2002;22:47-52.