成人肾恶性横纹肌样瘤11例临床分析

Eleven cases of adult malignant rhabdoid tumor of kidney: a clinical analysis

目的：观察成人肾恶性横纹肌样瘤的临床表现、治疗方法及预后。方法对11例成人肾恶性横纹肌样瘤患者的临床资料作回顾性分析。结果11例成人肾恶性横纹肌样瘤患者主要临床表现为腰腹疼痛4例，肉眼血尿2例，消瘦、乏力2例。 B超检查均提示肾占位性病变；CT检查均提示肾实质不规则混杂密度肿物影，肿瘤内有坏死液化，增强扫描均有不均匀强化改变。术后病理检查结果均确诊为肾恶性横纹肌样瘤。11例均行根治性肾切除术，术后辅以生物免疫治疗。根据NWTS3分期标准：Ⅰ期4例、Ⅱ期3例、Ⅲ期4例。4例术后1 a内死于肿瘤多器官转移；3例分别死于心肌梗死、脑出血、肾功能衰竭；3例术后随访7～26个月无瘤生存。结论成人肾恶性横纹肌样瘤临床表现无特异性，确诊依赖于病理学检查，应争取早期行根治性肾切除术和腹膜后淋巴结清扫术，Ⅰ期和Ⅱ期术后及时辅以生物免疫治疗疗效良好，Ⅲ期和Ⅳ期预后差。

Objective To observe the diagnosis , treatment and prognosis of adult malignant rhab doid tumor of the kidney .Methods The clinical data from 11 patients with adult malignant rhabdoid tumor of the kidney were retrospective-ly analyzed.Results The main clinical manifestations of 11 patients：waist pain was found in 4 cases, gross hematuria in 2 cases, weight loss and fatigue in 2 cases.B-ultrasonic examination showed all had renal lesions , computed tomography （ CT） revealed mixed density irregularities mixed with renal tumor shadow and liquefaction necrosis within the tumor , and enhanced scan showed that all had heterogeneous enhancement changing .Eleven cases underwent radical nephrectomy com-bined with postoperative immune therapy .According to the NWTS3 staging system, 4 cases had stageⅠtumor, 5 cases had stage Ⅱtumor and 2 cases had stage Ⅲtumor.Four cases died of multiple organ metastasis within 1 year after operation, 3 cases died of myocardial infarction , cerebral hemorrhage or renal failure , and 3 cases were follwed up for 7 to 26 months with disease-free survival .Conclusions The clinical manifestations of adult malignant rhabdoid tumor of the kidney have no specificity , the final diagnosis depends on pathological examination , and radical nephrectomy and retroperitoneal lymph node dissection should be done as early as possible .Immune therapy promptly after operation for stageⅠandⅡtumors re-sponds well for the prognosis , but poor efficacy for stage Ⅲand Ⅳtumors.