PEComa临床病理分析与影像诊断

目的:探讨血管周上皮样细胞肿瘤（PEComa）的临床特点及计算机体层成像（CT）、磁共振成像（MRI）表现，提高对PEComa的诊断准确率。方法:收集2021年12月30日中山市人民医院收治的经病理证实的1例肾脏恶性PEComa患者的影像及临床资料，结合搜索2000—2021年期间中国知网（CNKI）、万方数据库资料，...

Clinicopathological analysis and imaging diagnosis of PEComa

Objective　To investigate theclinical characteristics and CT and MRI manifestations of perivascularepithelioid cell tumor (PEComa), and to improve the diagnostic accuracy forPEComa. Methods　The imaging and clinical data of pathologically confirmed 1 renalmalignant PEComa patient admitted to Zhongshan City People's Hospital onDecember 30, 2021 were collected, and the location, size, morphology, boundary,density, and enhancement characteristics of tumor and its relationship withsurrounding tissues were analyzed combined with relevant literatures publishedin CNKI and Wanfang database from 2000 to 2021, and the imaging manifestationswere summarized. Results　The patient's tumor was located in the left kidney and underwentlaparoscopic radical resection of the lesion. The lesion was postoperativelypathologically confirmed as malignant PEComa. No metastasis or recurrence wasfound during the follow-up. A total of 239 patients were recruited from theliteratures, including 54 males (22.6%) and 185 females (77.4%), 20 cases ofwhom were clearly malignancy. The main clinical features were low back pain andlumbago, followed by unintentional discovery in physical examination, and therarest reasons were emergency diagnosis of tumor rupture and hemorrhage,hematuria, abdominal mass, and unintentional discovery of lesions in otherplaces. Pathologically, PEComas were composed of epithelioid cells from clearto eosinophilic cytoplasm distributed radially around blood vessels, usuallyexpressing melanocytes and myogenic markers, characterized by HMB45+. Imagingfeatures were visceral or retroperitoneal masses with well-defined boundaries,low to isodense density on plain scan, and mild/obvious uneven enhancement onenhanced scan. Conclusion　PEcoma is a rare disease, and imaging examination based on clinical,CT, MRI, and other imaging findings helps to improve the diagnostic accuracy.