Primary pulmonary perivascular epithelioid cell tumor:a case report and literature review / 肿瘤研究与临床

ABSTRACT

Objective To investigate the clinicopathological features,the diagnosis and therapy of primary pulmonary perivascular epithelioid cell tumor (PEComa). Methods One case of primary pulmonary perivascular epithelioid cell tumor diagnosed and treated in April 2010 was presented. The clinical symptoms,imaging, pathology features, treatment and follow-up record of the patient were analyzed retrospectively,and relevant literatures were reviewed. Results The patient had not special clinical symptoms and was found through the medical checkup. The imaging methods could easily find the tumor but hard to diagnose it. The final diagnosis depended on the pathologic features and immunohistochemistry techniques. Among present literatures, scarce cases of pulmonary malignant PEComa were reported. Conclusion Primary pulmonary PEComa is a very rare tumor that arises from mesenchymal tissues, especially the malignant cases. The diagnosis of PEComa mainly depends on the pathological features. Because of the uncertain biologic behavior pulmonary PEComa should be followed up closely for a long term.