成人原发性肾肉瘤的临床诊治

Diagnosis and treatment for primary adult renal sarcoma

目的探讨成人原发性肾肉瘤的诊断、治疗和预后。方法收集1985年-2009年收治的成人肾脏肿瘤1 654例,对其中17例原发性肾肉瘤进行回顾性分析。结果 17例患者中,10例以腹部肿块为首发症状。患者均行手术治疗。术后病理：平滑肌肉瘤7例,横纹肌肉瘤2例,恶性纤维组织细胞瘤2例,低分化肉瘤2例,脂肪肉瘤、纤维肉瘤、胚胎性横纹肌肉瘤及平滑肌肉瘤合并嫌色细胞瘤各1例。术中死亡1例,15例患者规律随访5-60个月,目前1例无瘤存活9个月,死亡14例,14例患者术后平均生存期18（5-60）个月;平滑肌肉瘤组28（11-60）个月,2例恶性纤维组织细胞瘤分别存活4个月和8个月。结论原发性肾肉瘤临床症状与晚期肾细胞癌类似,总体预后较差,肿瘤分期、病理类型及术后有无残余瘤是决定预后的重要因素。

Objective To investigate the clinical diagnosis,treatment and prognosis of primary adult renal sarcoma.Methods Totally 1 654 cases of adult renal tumors were treated from 1985 to 2009,17 cases of adult primary renal sarcoma in all of the tumors were analyzed retrospectively.Results The first symptom of 10 cases in all renal sarcomas was lumbar and abdominal mass.All 17 patients accepted radical nephrorectomy,with the pathological diagnosis of leiomyosarcoma（7 cases）,rhabdomyosarcoma（2 cases）,malignant fibrous histocytoma（2 cases）,low-differentiated sarcoma（2 cases）,and liposarcoma,fibrosarcoma,embryonic sarcoma and leiomyosarcoma coexist with chromophobe renal cell carcinoma for each one.One with tumor thrombosis in inferior vena cava died from pulmonary artery thrombosis.Fifteen cases were regularly followed up;one still lives without recurrence for 9 months,the other 14 cases died in 5 to 60 months with the average survival duration of 18 months.Average survival of leiomyosarcoma group was 28（11～60） months,and 2 case of malignant fibrous histocytoma died in 4 and 8 months respectively.Conclusion The primary renal sarcoma has the common clinical symptom similar with advanced renal cell carcinoma and poor prognosis.Stage of tumor,pathologic type and outcome of surgical clearence are the major prognostic factors.