肾间叶来源恶性肿瘤的临床特点及预后分析

Clinical features and prognosis of renal malignant mesenchymal tumors

目的探讨肾间叶来源恶性肿瘤(RMM)的临床特点、治疗方法及预后情况。方法回顾性分析2005年1月至2019年6月新疆医科大学第一附属医院收治的48例RMM患者的临床资料,男29例,女19例。汉族21例,少数民族27例。中位年龄55(16~79)岁。首诊主诉腰痛31例,腹胀8例,血尿2例;体检发现6例;1例因上臂痛于骨科就诊,完善检查提示为肾脏来源肿瘤。CT检查多表现为肾占位性病变,部分肿瘤可有液化、坏死或有囊性改变,增强后肿瘤不规则强化,边缘不清,与肾癌较难鉴别。肿瘤位于左侧30例,右侧18例。所有患者完善术前血常规、生化、凝血等检查。凝血酶原时间正常39例。根据初诊时的影像学结果进行临床分期:T_(1)期7例,T_(2)期16例,T_(3)期20例,T_(4)期5例。8例首次就诊时发现远处转移,分别为肺转移4例,腹膜后转移2例,骨转移2例。13例行穿刺活检以明确诊断。本组48例中,术前诊断为肾肿瘤29例,肾占位性病变12例,腹膜后占位7例。17例行根治性肾切除术,22例行肾部分切除术,9例穿刺活检明确诊断后未接受手术治疗。结果本组48例病理诊断为平滑肌肉瘤17例,脂肪肉瘤17例,其他类型间叶来源恶性肿瘤14例。平均随访38.8(7~180)个月,死亡30例,平均生存时间39个月。平滑肌肉瘤、脂肪肉瘤、其他类型肿瘤的5年生存率分别为18.2%、30.8%、50.1%,差异有统计学意义(P=0.047)。凝血酶原时间正常和异常患者5年生存率分别为36.8%和11.0%,差异有统计学意义(P=0.018)。术前穿刺活检和非穿刺活检患者5年生存率分别为27.4%和32.6%,差异无统计学意义(P=0.523);根治性手术和肾部分切除术患者5年生存率分别为44.6%和29.8%,差异有统计学意义(P<0.05)。T_(1)、T_(2)、T_(3)、T_(4)期患者5年生存率分别为40.0%、31.6%、35.4%、0,差异有统计学意义(P=0.020)。多因素分析结果显示,术前凝血酶原时间(P=0.013)、肿瘤临床T分期(P=0.030)、手术方式(P=0.006)是影响RMM患者预后的独立危险因素。结论RMM较罕见,恶性程度高,临床症状以腰痛最常见。术前行穿刺活检不影响RMM的预后。术前凝血酶原时间正常、初诊临床分期早期、行根治性手术的患者预后较好。肾脂肪肉瘤的预后优于肾平滑肌肉瘤。

Objective To discuss the clinical features,treatment and prognosis of renal mesenchymal malignancy.Methods Retrospective analysis was performed on the clinical data of 48 patients with renal mesenchymal malignancies admitted from January 2005 to June 2019.The patients'age ranged from 16-79 years,including 29 males and 19 females.There were 21 cases of Han nationality and 27 cases of ethnic minorities.The main complained of lumbago were 31 cases,bloating 8 cases,blood urine 2 cases,and 6 cases by physical examination.And one case was seen in orthopedics due to upper arm pain.The patient's completion of the examination indicated a tumor of kidney origin.CT examination mostly showed renal space-occupying lesions.After enhancement,the tumor was irregularly enhanced,with unclear edges,which was difficult to distinguish from renal carcinoma.The tumors were on the left in 30 cases and on the right in 18 cases.All patients perfected preoperative blood routine,biochemical,coagulation and other examinations.Prothrombin time was normal in 39 cases.Clinical staging was conducted according to the imaging results at the time of initial diagnosis.7 were in clinical Stage T_(1),16 were in Stage T_(2),20 were in stage T_(3),and 5 were in stage T_(4).Distant metastases were found in 8 cases,including 4 lung metastases,2 retroperitoneal metastases,and 2 bone metastases.13 patients underwent needle biopsy to confirm the diagnosis.Among the 48 cases in this group,29 cases were diagnosed as renal tumor,12 cases were diagnosed as renal space occupying lesions,and 7 cases were diagnosed as retroperitoneal space occupying.17 underwent radical nephrectomy,22 underwent partial nephrectomy,and 9 did not receive surgical treatment after needle biopsy.Results Among the 48 patients,Conventional pathology confirmed 17 cases as leiomyosarcoma,17 cases as liposarcoma,and 14 cases as other types of mesenchymal malignancies.Follow-up ranged from 7 to 180 months,with an average follow-up of 38.8 months.Thirty patients died,and the average survival time was 39 months.The 5-year survival rates of leiomyosarcoma,liposarcoma and other types of tumors were 18.2%,30.8%and 50.1%,and the difference was statistically significant(P=0.047).The 5-year survival rates of patients with normal and abnormal prothrombin time were 36.8%and 11.0%,the difference was statistically significant(P=0.018).The 5-year survival rates of radical surgery and partial nephrectomy were 44.6%and 29.8%,and the difference was statistically significant(P<0.05).The 5-year survival rates of T_(1),T_(2),T_(3) and T_(4) patients were 40.0%,31.6%,35.4%and 0,and the differences were statistically significant(P=0.020).Multivariate analysis showed that preoperative prothrombin time(P=0.013),clinical T stage(P=0.030)and surgical method(P=0.006)were independent factors affecting the prognosis of patients with RMM.Conclusions Renal mesenchymal malignant tumor is rare,and highly malignant.Preoperative needle biopsy did not affect the prognosis of RMM.Patients with normal prothrombin time,early clinical staging and radical surgery had better prognosis.Renal liposarcoma has a better prognosis than renal leiomyosarcoma.