A Case of Primary Pulmonary Artery Intimal Sarcoma

Abstract

A 28-year-old Japanese woman complained of right chest pain, fever, and cough. Chest radiography revealed consolidation in her right lower lobe with a pleural effusion. She was diagnosed with pneumonia and treated with antibiotics, but her condition did not improve. She was referred to our hospital for evaluation. Contrast-enhanced computed tomography (CT) demonstrated a large saddle filling defect extending into the right pulmonary artery as well as the proximal lower lobe pulmonary artery branch. Massive pulmonary artery thromboembolism was suspected, and early surgical intervention was performed. Myxomatous soft tissue was located in the pulmonary artery trunk. We were unable to remove this tumor. Histologically, the tumor was a malignant spindle cell neoplasm, and was positive for vimentin but negative for CD34, desmin, MDM2 and cytokeratin’s (AE1/AE3). Based on the clinical presentation, radiological findings, and immunohistochemical staining, pulmonary artery intimal sarcoma was diagnosed. Our patient did not agree to more aggressive therapy because there is no established chemotherapy or other aggressive treatment and she desired pregnancy. Unfortunately, regrowth at the surgical margin of right pulmonary artery and multiple lung metastases were detected 3 months after surgery, the patient received chemotherapy with doxorubicin. But we didn’t control the disease activity, next chemotherapeutic agents or challenging therapy such as multi-targeted tyrosine kinase inhibitor will be administrated.