Study objectives: To define characteristic clinical and radiographic presentations of patients found to have primary sarcoma of the pulmonary artery and differentiate this entity from acute and chronic pulmonary thromboembolism. To review pathologic characteristics of pulmonary artery sarcoma.
Design: A retrospective review of medical records at a large medical center and a review of the literature.
Setting: Tertiary care medical center.
Patients: Patients found to have pulmonary artery sarcoma based on surgical or autopsy findings.
Interventions: Review of medical records.
Measurements and results: Presenting symptoms of patients with pulmonary artery sarcoma are similar to those of patients with other pulmonary vascular disease, including progressive dyspnea, cough, hemoptysis, and chest pain. Symptoms not commonly associated with pulmonary vascular disease consist of progressive weight loss, anemia, and fever, which are symptoms commonly associated with malignancy. The diagnosis is often not suspected prior to surgery, which is often undertaken with an alternative diagnosis such as bronchogenic carcinoma or chronic pulmonary thromboembolism. Prolonged survival is rare; most patients survive only a few months. Survival longer than 3.5 years was not observed. Recent experience with MRI of the thorax and transesophageal echocardiography suggests that these diagnostic entities may be rewarding in separating the abnormalities seen on chest radiographs and angiography from thrombus in the pulmonary artery, mediastinal adenopathy, or primary bronchogenic carcinoma.
Conclusions: Although a rare entity, the key is to suspect the presence of pulmonary artery sarcoma in evaluation of patients with atypical presentations of pulmonary vascular disease especially if associated with symptoms such as weight loss, fever, or anemia.