血管周上皮样细胞肿瘤的研究进展

The progress of perivascular epithelioid cell tumors

血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComas)是源于间质的罕见肿瘤,肿瘤细胞由独特的血管周上皮样细胞构成,均表达黑色素瘤标志物及黑色素特异性抗体。主要患病人群为女性,临床表现无明显特异性,可累及身体的各个部位,但起源于子宫的较多。良性肿瘤较多,复发及转移性少见,预后良好,手术切除为主要治疗方式,可辅以化疗及靶向治疗。我们基于已发表的文献进行了全面的调查,对PEComas的临床病理特征、分子生物学标记、诊断和鉴别诊断与治疗进行综述。

Perivascular epithelioid cell tumors(PEComas)are very rare mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells.The main patient group is female,and there is no obvious specificity in clinical manifestations.It can affect all parts of the body,but it originates in the uterus.At present,surgical excision is thought to be the most direct and effective treatment,then followed by adjuvant radiotherapy and chemotherapy.We conducted a comprehensive investigation based on the published literature to review the clinicopathological characteristics,molecular biological markers,diagnosis and differential diagnosis and treatment of PEComas.