肾血管周上皮样细胞肿瘤10例临床分析

Clinical analysis of 10 cases of renal perivascular epithelioid cell tumor

目的探讨肾血管周上皮样细胞肿瘤(PEComa)的临床特点。方法回顾性分析2013年1月至2020年12月中山大学附属第一医院收治的10例肾PEComa患者的临床资料。所有患者均经外科手术治疗,术后标本均行病理学及免疫组化检测,术后随访采用门诊及电话随访相结合。结果10例患者中,5例行肾部分切除术,5例行根治性肾切除术。10例患者最终病理诊断均为肾PEComa,8例免疫组化染色检查示HMB45阳性,8例Melan-A阳性,8例Actin灶性阳性,6例Vimentin阳性,2例Desmin阳性,1例CD34阳性,1例S-100阳性,Ki-67呈不同程度表达(1%~5%)考虑为阴性。10例患者随访时间16~78个月,除1例患者不明原因死亡外,其余患者均未见转移或复发。结论肾PEComa临床上少见,特征不明显,影像学检查难以确诊,病理为诊断金标准,行手术切除后大多数均预后较好。

Objective To investigate the clinical features of perivascular epithelioid cell tumor(PEComa).Methods The clinical data of 10 patients with renal PEComa admitted to the First Affiliated Hospital of Sun Yat-sen University during Jan.2013 and Dec.2020 were retrospectively analyzed.All patients underwent surgical treatment.Pathological and immunohistochemical examinations were performed on postoperative specimens.Postoperative follow-up was conducted by outpatient department and telephone.Results Partial nephrectomy was performed in 5 cases,and radical nephrectomy in 5 cases.The final pathological diagnosis of all patients was renal PEComa.Immunohistochemical staining showed HMB45 positive in 8 cases,Melan-A positive in 8 cases,focal Actin positive in 8 cases,Vimentin positive in 6 cases,Desmin positive in 2 cases,CD34 positive in 1 case,and S-100 positive in 1 case.Ki-67 was expressed in different degrees(1%-5%).During the follow-up of 16 to 78 months,1 patient died of unknown causes,and no metastases or recurrences were observed.Conclusion As a rare disease,renal PEComa has no obvious features and is difficult to be diagnosed by imaging examination.Pathology is the gold standard for diagnosis,and most patients have good prognosis after surgical resection.