原发性肾平滑肌肉瘤13例临床病理分析

目的分析13例肾脏平滑肌肉瘤患者的生存情况,评估预后相关因素。方法回顾性分析1996年至2008年间我院诊治的13例肾脏平滑肌肉瘤患者的临床症状、病理学特征、治疗方式及随访资料,运用logistic回归评估各项观察指标与预后的相关性,分析各指标对预后的影响。结果患者平均年龄为45.7岁,61.5%(8/13)为女性。平均肿瘤直径为〔11.1±10.2(3.0～34.0)〕cm,53.9%(7/13)累及肾包膜,23.1%(3/13)淋巴结阳性,完成随访11例,中位生存时间为39.2月(9～81月),1年、3年、5年生存率分别为81.8%,54.5%、16.2%。8例患者出现远处转移,截止本次随访9例患者死亡。相关性分析显示:患者的生存与年龄、病理分级、是否完整切除、是否淋巴结转移、是否侵犯包膜或肾盂、是否化疗等无相关性。结论原发性肾脏平滑肌肉瘤为高致死率的肾脏恶性肿瘤,需尽早发现、手术切除,否则生存预后差。

Primary leiomyosarcoma of the kidney: a clinicopathological study of 13 cases at a high-volume institution

Objective To analyze the clinical characteristics,treatment,and survival of adult patients with renal leiomyosarcoma treated at our institution during the past 13 years.Methods A retrospective review was performed to collect the demographic distribution,clinical manifestation,treatment,and outcome data about 13 adult patients of renal leiomyosarcoma treated at our institution from January 1996 to January 2008.The clinical parameters were analyzed to determine the effects on survival by logistic regression.Results Mean age at diagnosis was 45.7 years(range 20 to 71),and 61.5%(8/13) were female.Mean tumor diameter was(11.1±10.2) cm(range 3 to 34 cm),53.9%(7/13) had direct invasion of renal capsule,and 23.1%(3/13) had lymphovascular invasion. The followup information of 11 cases was available,and duration of follow-up was an average of 39.2(range 9-81) months.The overall survival(OS) rate at 1,3,and 5 years was 81.8%,54.5%,and 16.2% respectively.8(72.7%) patients developed metastasis,and 9(81.8%) patients eventually died.The survival of the patients did not show correlations with age,pathologic grade,extrarenal component,lymphovascular invasion,complete resection or not and chemotherapy or not.Conclusion Primary renal leiomyosarcoma has a poor survival prognosis regardless of the underlying histological and clinical features.