Primary pulmonary angiosarcoma

Primary pulmonary angiosarcoma is a rare type of malignant vascular tumour with poor prognosis. Diagnosis is often late due to non-specific symptoms and low clinical suspicion for angiosarcoma. A 72-year-old man presented to hospital with a 6-month history of mild progressive dyspnoea, with associated cough, episodes of presyncope and weight loss. CT pulmonary angiogram (CTPA) was reported as a large saddle pulmonary embolism extending into both the right and left pulmonary arteries. Further Multidisciplinary team meeting (MDM) discussion, and review of CTPA and subsequent investigations revealed a large primary pulmonary artery sarcoma which was later confirmed histology. The patient was referred to the cardiothoracic surgeons and underwent left radical pneumonectomy.

Giant Pulmonary Artery Thrombotic Material, Due to Chronic Thromboembolic Pulmonary Hypertension, Mimics Pulmonary Artery Sarcoma

In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery sarcoma (PAS) due to extensive filling defects and extraluminal findings. Although surgery was postponed for nine months due to the COVID-19 pandemic, CTEPH diagnosis, due to a high-thrombus burden, was finally confirmed after pulmonary endarterectomy (PEA). Conclusively, imaging findings of rare cases of CTEPH might mimic PAS and the surgical removal of the lesion are both needed for a final diagnosis. What is Already Known about This Topic? Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy, which originates from the intimal layer of the pulmonary artery (PA); Chronic thromboembolic pulmonary hypertension (CTEPH) is based on chronic, organized flow-limiting thrombi inside PA circulation and subsequent pulmonary hypertension. What Does This Study Contribute? Since radiological findings of CTEPH cases might rarely mimic PAS, pulmonary artery endarterectomy and subsequent histopathologic study are needed for a final diagnosis.