Pulmonary artery intimal sarcoma: clinical and pathological characteristics
JIANG Yi-na
ZHANG Guan-jun
YANG Zhe
Abstract：Purpose To investigate the clinicopathlogical characteristics and molecular genetic alterations of pulmonary artery intimal sarcoma.Methods One case of pulmonary artery intimal sarcoma was studied by immunohistochemical staining.The related literature was reviewed and summarized.Results The tumor was located in the lumen of pulmonary artery.Histopathologically,this case typically displayed a proliferation of spindle cells and in a myxoid background.Most of the spindle cells had significant anaplastic feature.Immunohistochemical stains showed that tumor cells were positive for vimentin.In addition,there were focally positive for SMA,desmin and MDM2.However,the other markers of specific differentiation,such as CK,CD117,ALK,CD34,CD31,myosin,myoglobin,F8 and EGFR were negative.Conclusions Pulmonary artery intimal sarcoma originates from the arterial intima of elastic pulmonary arteries.It is a malignant mesenchymal tumor with poor prognosis.There is no typical clinical symptom and distinctive immunophenotype.A diagnosis can be made by the location and histopathological features.
Keywords：Pulmonary arteryIntimal sarcomaImmunohistochemistryGenetic profile
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 194-197 )