原发性肾脏恶性血管周上皮样细胞肿瘤的临床分析

摘要

目的 总结原发性肾脏恶性血管周上皮样细胞肿瘤(PEComa)的临床特点及预后.方法 回顾性分析2013年8月至2017年6月我院收治的8例原发性肾脏恶性PEComa患者的临床资料.男4例,女4例.年龄27～ 65岁,平均46.3岁.所有肿瘤均为单发,肿瘤最大径1.8～17.2cm,平均9.4 cm.3例因体检发现肾肿瘤,3例间歇性腰痛并发热,1例肉眼血尿,1例因右手右足软组织红肿疼痛就诊.术前泌尿系CT或MRI检查均提示肾肿瘤.术前均诊断为肾脏恶性肿瘤,其中2例合并下腔静脉癌栓.1例肺、肝、骨等多处转移.临床分期:T1N0 M0期2例,T1N0M1期1例,T2N0 M0期2例,T3M0M0期1例,T4N0M1期2例.3例行根治性肾切除术,1例行肾动脉化疗栓塞术+根治性肾切除术,2例行肾部分切除术,2例行根治性肾切除术+下腔静脉癌栓取出术.1例因术前发现远处多发转移,行根治性肾切除术后予全身异环磷酰胺+表柔比星化疗;1例因肿瘤直径过大,术前行奥沙利铂局部栓塞化疗.余均未行辅助治疗.结果 术后病理检查肿瘤切面呈灰红、灰黄色,多结节状,质软,部分有不完整包膜.镜下可见较大的非典型性细胞,部分呈梭形,细胞排列紊乱,部分合并大量坏死组织,胞质丰富浅嗜伊红色.组织成分可见厚壁血管、平滑肌样细胞、脂肪组织等.免疫组化染色检查结果示,8例中4例HMB45阳性,4例Melan-A阳性,7例平滑肌肌动蛋白(SMA)阳性,7例细胞角蛋白(CK)阴性,6例S-100阴性,Ki-67呈不同程度表达(10％ ～ 70％).术后病理诊断均为肾脏恶性PEComa.8例术后随访14～60个月,平均29个月.1例术前已发生多发转移者,术后25个月死于恶病质;1例术后6个月出现多发转移(肺、骨、肝),术后20个月死于恶病质.1例肿瘤局部复发,带瘤生存.余未见复发转移.结论 原发性肾脏恶性PEComa临床罕见,肿瘤直径往往较大,确诊依靠病理检查结果.治疗首选患侧根治性肾切除术,术后易发生复发、远处转移.%Objective To summary the pathological and clinical characteristics,treatments and prognosis of malignant renal perivascular epithelioid cell tumor (PEComa).Methods Between August 2013 and June 2017,8 cases of malignant renal PEComa were analysed respectively.Of all the patients,there were 4 males and 4 females,aged 27-65 years with the average of 46.3 years old.Three cases were detected in routine examination occasionally,three cases complained of intermittent back pain with fever,one presented with swollen and painful right hand and right foot,and one case presented with hematuria.CT or MRI examinations indicated the malignant lesions before the surgery.Clinical staging was performed with 2 cases of T1N0M0,1 case of T1 N0M1,2 cases of T2N0M0,1 case of T3M0M0,and 2 cases of T4N0M1.Three cases underwent radical nephrectomy,1 case underwent radical nephrectomy plus renal artery embolization,2 cases underwent partial nephrectomy,and 2 cases underwent nephrectomy plus inferior vena cava tumor thrombectomy.One case was treated with ifosfamide plus epirubicin after operation due to multiple distant metastases and 1 case was treated with oxaliplatin before the sugery because of excessive tumor diameter.None of the rest received any adjuvant therapy.Results Postoperative histopathological examination showed multiple nodules in reddish gray and yellow color,with soft texture and partial incomplete capsule.Microscopically,there were large atypical cell components,some of which were spindle shaped,with disordered cell arrangement,some of which were associated with a large amount of necrotic tissue,and abundant light eosinophil cytoplasm.Tissue components can be found thick-wall blood vessels,smooth muscle-like cells,and adipose tissue.Immunohistochemistry showed 4 cases were positive of HMB-45,4 of Melan-A,7 of SAM.Seven cases were negative of CK,6 of S-100 75％ (6/8) and Ki67 (10％-70％).Two cases died of multiple metastases (lung,bone,liver),1 case survived with tumor recurrence,with a follow-up from 14 to 60 months (mean 29 months).Conclusions Primary malignant renal PEComa is rare in practice with relative large diameter.The diagnosis depends on pathological findings,and radical nephrectomy is the first choice of treatment.It is easy to recur and metastasize after the operation.