Primary pulmonaryperivascular epithelioid cell tumor:a case report and literature review

：Objective To investigate the clinicopathological features,thediagnosis and therapy of primary pulmonary perivascular epithelioid cell tumor (PEComa).Methods One case of primary pulmonary perivascular epithelioid cell tumor diagnosed andtreated in April 2010 was presented. The clinical symptoms,imaging, pathology features,treatment and follow-up record of the patient were analyzed retrospectively,and relevantliteratures were reviewed. Results The patient had not special clinical symptoms and wasfound through the medical checkup. The imaging methods could easily find the tumor buthard to diagnose it. The final diagnosis depended on the pathologic features andimmunohistochemistry techniques. Among present literatures, scarce cases of pulmonarymalignant PEComa were reported. Conclusion Primary pulmonary PEComa is a very rare tumorthat arises from mesenchymal tissues, especially the malignant cases. The diagnosis ofPEComa mainly depends on the pathological features. Because of the uncertain biologicbehavior pulmonary PEComa should be followed up closely for a long term. Key words: Pulmonary;  Perivascular epithelioid cell tumor