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目的:探讨胰腺血管周上皮细胞肿瘤(PEComa)的影像学表现及临床病理特征。方法:收集2013年11月至2017年11月间海军军医大学附属长海医院收治的胰腺PEComa患者的临床资料，检索胰腺PEComa相关文献，分析PEComa相关的影像学表现及临床病理特征。结果:长海医院收治胰腺PEComa 2例，文献报道胰腺PEComa 29例，共31例，其中男性3例，女性27例，1例未提及性别。发病年龄为17～74岁，平均48岁。20例病灶位于胰头颈部，11例位于胰体尾部。27例单发，4例多发。病灶长径为1.0～11.5 cm，平均4.1 cm。对病灶囊实性进行描述的23例患者中，18例为实性。对胰管进行描述的21例患者中，14例胰管无扩张。对病灶强化方式进行描述的21例患者中，16例强化程度高于胰腺。结论:胰腺PEComas多发生于胰头颈部，病灶偏小，常向胰腺轮廓外突出，边界清，包膜完整，实性多见，增强后强化程度常高于胰腺。“,”Objective:To evaluate the imaging and clinicopathological characteristics of pancreas perivascular epithelioid cell tumors (PEComa).Methods:The clinical data of patients with pancreatic PEComa admitted in Changhai Hospital of Navy Medical University from Nov 2013 to Nov 2017 were retrospectively analyzed. Related literature on PEComa were searched and the image and clinicopathological characteristics were investigated.Results:Two pancreatic PEComa patients were admitted in Changhai Hospital. Twenty-nine PEComa patients were reported in previous literature. A total of 31 cases were detected, including 3 men and 27 women. The onset age of the patients ranged from 17-74 years old with a mean of 48 years old. The lesions were located at the pancreatic head and neck in 20 cases, and at the pancreatic body and tail in 11 cases. 27 cases had a single lesion, and 4 cases had multiple lesions. Tumor diameter ranged from 1.0-11.5 cm with a mean of 4.1 cm. The cystic solid of the lesions was recorded in 23 patients, and 18 cases had solid mass. Pancreatic duct dilation was reported in 21 patients and 14 of them did not have pancreatic duct dilation. Enhancement mode of the lesions was examined in 21 patients, and the enhancement degree of the lesions were higher than that of the pancreas in 16 cases.Conclusions:PEComa has a certain imaging characteristic, which could help obtain a preoperative diagnosis.