原发性腹膜后血管周上皮样细胞瘤临床病理及影像特征分析

An Analysis of the Clinicopathological and Imaging Features of Primary Retroperitoneal Perivascular Epithelioid Cell Tumor

目的探讨原发性腹膜后PEComa临床病理及影像特征。方法回顾性分析原发性腹膜后PEComa 1例并结合2007~2017年文献报道,对其特征进行总结、归纳。结果腹膜后PEComa好发于中年女性(52%),大多数患者就诊前有腹痛或腹部不适病史(74%),好发部位以盆腔多见(43%),中腹部次之(39%)。影像检查以实性肿块为主(65%),囊性及囊实性肿块次之(35%),轮廓多半较清晰光整(61%),平均直径为(11±5) cm。腹膜后PEComa以恶性为主(57%),其次为性质待定(39%),良性少见(4%)。肿瘤细胞多表达HMB-45 (100%)、Vimentin (88%)、SMA(85%)、Melan-A(67%)、Desmin(62%)等,临床治疗多采用手术切除(78%)。结论原发性腹膜后PEComa有一定的临床及影像表现,但缺乏特异性,确诊依赖病理诊断,因其生物学行为不明确,建议尽早行手术切除并术后长期随访。

Objective To investigate the clinicopathological,imaging features of the primary retroperitoneal perivascular epithelioid cell tumor(PEComa).Methods A retrospective analysis of 1 case of primary retroperitoneal PEComa was performed,and combining literature reported from 2007 to 2017 for summing up its characteristics.Results Primary retroperitoneal PEComa was commonly seen in middle-aged women(52%).Most patients had a history of abdominal pain or abdominal discomfort before treatment(74%),while pelvic cavity was the predilection site(43%),and the second was midabdomen(39%).The tumor could be showed solid,cystic-solid and cystic in imaging examination,and most were solid(65%),the contour was mostly clear(61%),with an average diameter of(11±5)cm.Primary retroperitoneal PEComa was mainly malignant(57%),followed by undetermined(39%)and was rare in benign(4%).The tumor cells expressed HMB-45(100%),Vimentin(88%),SMA(85%),Melan-A(67%)and desmin(62%),etc.Surgical resection was often used in clinical treatment(78%).Conclusion There were certain characteristics of clinical and imaging performance for primary retroperitoneal PEComa.However,lacking specificity of clinical and imaging,so final diagnosis should depended on pathological.For uncertain biological behavior,surgical resection is recommended as early as possible,and with long-term follow-up after operation.