| Objective:To review the clinical characteristics, auxiliary examinations, diagnosis and related therapy of primary pulmonary artery leiomyosarcoma thus to improve the comprehension of such disease.Methods:A PPAL case presenting as dry cough and Chest tightness after activities was reported, which pathologically confirmed via EBUS-TBNA. Literature review was carried out via "lung" and "pulmonary artery, leiomyosarcoma", as key words separately in CKNI, China info and Pubmed Database.Result:A 47-years old female was admitted into Qilu Hospital, Shandong University, for the complaints of "dry cough and Chest tightness after activities for six months". The admitting diagnosis was made when we analyzing the assistant examinations before. Assistant examinations such as (1,3)-β-D-glucan test, Galactomannan serum index test, T-SPOT,ANCA are Negative. The arteriovenous ultrasonic of lower limbs shows no positive finding. PET-CT scans also indicated high uptake ratio of 18-Fluoro-2-deoxyglucose (18-FDG) of the mass in pulmonary artery.The patient underwent bronchoscope for 2 times after admitting into our hospital. And was finally diagnosed as PPAL bypass EBUS-TBNA. Then the patient was transferred into Department of Chemotherapy for further treatment. Totally 8089 patients from 1225 essays CKNI clinical and China info analysis and 1198 essays are gotten.58 cases are PPAL.There are 23 male and 35 female, with the mean age 52.5±12.5 year-old. Common syndrome as Chest tightness after activities, cough, chest pain, hemoptysis, syncope are reported. Most cases of pulmonary artery tumors were diagnosed with surgery or during postmortem examination. About 78 percent was misdiagnosed as pulmonary embolism.Conclusion:Pulmonary primary leiomyosarcoma is a rare pulmonary malignant tumor, with no specific syndrome. It is often misdiagnosed as pulmonary embolism.The differential diagnosis between PPAL and PE should be pay more attention to. Surgery is recommended the first selection therapy. |
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