肾血管周上皮样细胞瘤诊疗研究进展

Progress in diagnosis and treatment of renal perivascular epithelioid cell tumor

肾血管周上皮样细胞瘤(renal perivascular epithelioid cell tumor,PEComa)是起源于间质的罕见肿瘤,由独特的血管周上皮细胞组成,可累及身体多部位。诊断肾PEComa主要依靠病理学免疫组化,通过黑素细胞标记物和肌源性标记物的独特表达进行识别。其主要治疗方式为手术切除,预后良好。本文对肾PEComa的细胞组织来源、临床病理特点、诊断与治疗相关进展作一综述,旨在提供对这一罕见疾病的深入了解。

Renal perivascular epithelioid cell tumor(PEComa) is a rare tumor originating from the interstitium,composed of distinctive perivascular epithelioid cells and can affect multiple parts of the body.PEComa is diagnosed primarily through pathological immunohistochemistry,identifying it by the unique expression of melanocytic and myogenic markers.The main treatment for renal PEComa is surgical resection,and the prognosis is good.This article reviews the cellular tissue origin,clinical pathological features,diagnostic,and therapeutic advances of renal PEComa,aiming to provide a deeper understanding of this rare disease.