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 the clinical characteristics and CT and MRI manifestations of perivascular epithelioid cell tumor (PEComa), and to improve the diagnostic accuracy for PEComa. Methods　The imaging and clinical data of pathologically confirmed 1 renal malignant PEComa patient admitted to Zhongshan City People's Hospital on December 30, 2021 were collected, and the location, size, morphology, boundary, density, and enhancement characteristics of tumor and its relationship with surrounding tissues were analyzed combined with relevant literatures published in CNKI and Wanfang database from 2000 to 2021, and the imaging manifestations were summarized. Results　The patient's tumor was located in the left kidney and underwent laparoscopic radical resection of the lesion. The lesion was postoperatively pathologically confirmed as malignant PEComa. No metastasis or recurrence was found during the follow-up. A total of 239 patients were recruited from the literatures, including 54 males (22.6%) and 185 females (77.4%), 20 cases of whom were clearly malignancy. The main clinical features were low back pain and lumbago, followed by unintentional discovery in physical examination, and the rarest reasons were emergency diagnosis of tumor rupture and hemorrhage, hematuria, abdominal mass, and unintentional discovery of lesions in other places. Pathologically, PEComas were composed of epithelioid cells from clear to eosinophilic cytoplasm distributed radially around blood vessels, usually expressing melanocytes and myogenic markers, characterized by HMB45+. Imaging features were visceral or retroperitoneal masses with well-defined boundaries, low to isodense density on plain scan, and mild/obvious uneven enhancement on enhanced 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.