Abstract
A computed tomography (CT) thorax demonstrated abnormal opacification within the right pulmonary artery (RPA). The patient was initially diagnosed with PE and anticoagulation treatment commenced. Despite anticoagulation both the symptoms and radiological abnormalities were unchanged 1 month later and she was referred to a tertiary centre for evaluation. Positron emission tomography computed tomography (PET-CT) revealed abnormal uptake within the RPA. Pulmonary angiogram confirmed an obstructing lesion, from which aspiration tissue sampling confirmed a high-grade pleomorphic sarcoma of the pulmonary artery. The patient underwent a right pneumonectomy followed by adjuvant chemotherapy. Conclusion: Pulmonary artery sarcomas are rare tumours with only a few hundred reported cases. Delay in diagnosis is frequent with PE the most common misdiagnosis. Clinical features that may raise suspicions of pulmonary artery sarcoma in patients with suspected PE include fever, weight loss, clubbing and elevated inflammatory markers such as CRP. Surgical resection as part of multi-modality therapy has improved survival for patients from an average of 1.5 months without treatment to approximately 3 years in the largest analysis of outcomes of this rare tumour. Disclosure: All authors have declared no conflicts of interest.
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