BACKGROUND: Primary pulmonary artery sarcoma (PPAS) is a rare tumor that mimics pulmonary thromboembolism (PE). Similarities to PE can delay the diagnosis and misguide the treatment of PPAS. This study aimed to evaluate tumor characteristics and outcome predictors among those diagnosed with PPAS and misdiagnosed as PE. METHODS: From 1991-2010, 10 PPAS cases were available from the Cleveland Clinic (CC) institutional database and another 381 cases were reported in the literature. Patient characteristics, tumor subtypes, diagnostic testing & timing, interventions and clinical outcomes were analyzed. We also noted effects of misdiagnosis as PE and clinical outcome as a result of inappropriate intervention. RESULTS: Among 391 confirmed cases of PPAS, the mean age at diagnosis was 52±14 years; 55% were male. The median duration of symptoms prior to diagnosis was 100 [interquartile range (IQR), 30-210] days. Nearly half (47%) of PPAS were originally misdiagnosed as PE including 39% that received thrombolytic and/or anticoagulation therapy. For every doubling of time from symptom onset to diagnosis, the odds of death increased by 46% (OR: 1.46, 95% CI: 1.21-1.82; P<0.001). The odds of death (OR: 2.66, 95% CI: 1.58-4.54; P=0.0003) and occurrence of distant metastasis (OR: 2.30, 95% CI: 1.30-4.15; P=0.049) were increased among those who did not receive chemotherapy but chemotherapy did not impact local recurrence. Those with complete resection had a better survival. CONCLUSIONS: PPAS has a radiological appearance similar to PE, which makes accurate and timely diagnosis challenging. More rapid diagnosis may lead to earlier, appropriate surgical treatment and improved outcomes, when combined with adjuvant treatment.