Clinical Analysis Of Five Patients With Pulmonary Artery Sarcoma And Literature Review

ObjectiveTo explore the characteristics of the clinical manifestations,laboratory examination,imaging,and relevant treatment of pulmonary artery sarcoma(PAS),improve the clinical diagnosis and treatment level of PAS,thus to make more patients benefit from it.MethodsThe clinical data of five patients with PAS confirmed in Sir Run Run Shaw Hospital,Zhejiang University School of Medicine,from September 2013 to April 2017 were retrospectively analyzed,and relative literatures were reviewed for discussion about their clinical features and hospital course.Results(1)Among the five patients with PAS,the ratio of male to female was 3:2,39-58 years old,with an average of 50.80±7.83 years old.(2)One patient with acute onset,one patient with subacute onset,and three patients with chronic onset.The main clinical manifestations were chest tightness(3/5),tachypnea(3/5),weight loss(2/5),chest pain(1/5),cough(1/5),fever(1/5),emesis(1/5)and stomachache(1/5).One patient was found jugular venous distention,and the others were not significantly abnormal.(3)Among the five patients,the d-dimer and hs-CRP were all high,the serum albumin was subnormal,and two patients' ESR indicated a high level,and one patient' s NT-proBNP level was high.The tumor markers were examined in five patients,two of which increased with NSE,and two increased with CA125.(4)Five patients underwent echocardiography,three of them found mass samples within the pulmonary artery,the rest had different degree of pulmonary hypertension(3/5),tricuspid regurgitation(4/5),enlarged right heart(2/5),pericardial effusion(1/5),etc.Four patients' chest X-ray examination showed increased lung gate shadow(3/4),pulmonary artery protrusion(1/4),and lung occupying(l/4).Two patients with lung ventilation/perfusion imaging showed insufficient blood perfusion and did not match the ventilation imaging.CTPA examination of five patients were found filling defect,involving the pulmonary artery trunk(4/5),left pulmonary artery(4/5),right pulmonary artery(3/5),as well as the left and right pulmonary artery branch,one case showed mild-to-moderate inhomogeneous enhancement within the lump.Four patients underwent PET-CT examination,indicating that the metabolic rate of FDG was increased.(5)One patient underwent CT-guided needle aspiration biopsy and diagnosed PAS by pathology.One patient underwent ultrasound-guided percutaneous biopsy in the left pulmonary artery through bronchoscope,and the pathology revealed a large number of blood clots and a small number of chronic inflammatory cells.All patients underwent surgical treatment and were confirmed pulmonary artery sarcoma by pathology finally.ConclusionsPAS is a high malignancy with insidious onset There is no obvious specificity of its clinical symptoms,signs and general imaging manifestations.The diagnosis consciousness of this disease should be strengthened clinically.When highly suspecting PAS,we should take full advantage of CTPA and PET-CT examination,take pathological biopsy and surgical treatment,adjuvant radiation or chemotherapy if necessary;as early as possible,to prolong patient's survival time.