A case report of primary pulmonary artery intimal sarcoma

BACKGROUND: Pulmonary artery intimal sarcoma (PAS) is a very rare disease, its prevalence is about 0.001-0.003%. 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 where the symptom 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 histopathology. In this case, the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similar 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 an adjuvant treatment. CONCLUSION: We report a very rare case of pulmonary artery intimal sarcoma. Due to late diagnosis and delayed treatment in this case, the patient displayed a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.