Abstract
Background: Pulmonary artery intimal sarcoma(PAS) is a very rare disease, the prevalence of it is about 0.001%-0.003%1. PAS is often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus early diagnosis is very crucial and may improve patient outcome.Case presentation: Here we report a case in a Chinese male which the symptom as presentation was episodes of shortness of breath. Transthoracic echocardiography showed a solid mass in the pulmonary valve orifice, which was demonstrated to be a pulmonary artery intimal sarcoma diagnosed by histopathology2. In this case the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similiar to the pulmonary embolism. Half of them may be misdiagnosed as pulmonary embolism. Imaging studies are very helpful. Ultrasound and CT are the best due to their resolution and ability to assess the relationship of the mass with the surrounding structures. The final diagnosis is mostly made after surgical excision and this is the most effective treatment. At the same time, radiotherapy and chemotherapy after surgery is also a adjuvant treatment3.Conclusion: We report a very rare case of pulmonary artery intimal sarcoma, due to late diagnosis and delayed treatment in this case, the patient display a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.
A Case of Unresectable Pulmonary Artery Intimal Sarcoma with Prolonged Survival by Chemotherapy
