Pulmonary artery sarcoma: an insidious tumor still diagnosed too late. Analysis of the literature and report of a case.

Pulmonary artery sarcoma is an exceptionally rare tumor which must be considered in the differential diagnosis of pulmonary thromboembolism. We report the case of a 36-year-old woman and review 100 cases published in the literature between 1988 and 2005. The patient presented with a history of dyspnea, fatigue, fever, night sweats and anemia that did not respond to antibiotic therapy. She also had hemoptysis. Transvenous catheter biopsy was indicative of sarcoma. A left pneumonectomy was performed, followed by five cycles of chemotherapy. Histological and immunohistochemical studies documented an intimal sarcoma with myofibroblastic differentiation. The patient is alive and well 20 months after surgery. The clinico-pathological features of pulmonary artery sarcoma are described.