Dotan Zohar A, Tal Raanan, Golijanin Dragan, Snyder Mark E, Antonescu Cristina, Brennan Murray F, Russo Paul
Department of Urology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
J Urol. 2006 Nov;176(5):2033-8; discussion 2038-9. doi: 10.1016/j.juro.2006.07.021.
Urological sarcomas are rare. We describe a continued single institutional experience during 25 years.
The records from July 1977 to July 2003 were reviewed of all patients at our institution with sarcoma arising in the urinary tract or male genital system who were 16 years or older at diagnosis.
The primary tumor site in 131 patients was the bladder in 20, the kidney in 26, paratesticular in 57, the prostate in 21 and other in 7. Median followup was 4.0 years. The most common histological subtypes were leiomyosarcoma in 29% of cases and liposarcoma in 26%. Median tumor size was 7 cm and 78% of lesions were high grade. Of the patients 28 (21%) presented with metastatic disease and their median survival was 1.4 years compared to 10.7 years in patients without metastatic disease (p < 0.0001). Disease specific survival was 56% and 42% at 5 and 10 years, respectively and median survival was 7.6 years. On univariate analysis unfavorable prognostic variables for disease specific survival were metastasis at presentation, high tumor grade, a lack of leiomyosarcoma and liposarcoma histological subtypes, prostate sarcoma and large tumor size, incomplete surgical resection and positive surgical margin. Patient sex, age and surgical margin status were not significant predictors. On multivariate analysis 2 variables remained significant predictors of disease specific survival, including tumor size (HR 1.1, 95% CI 1.02 to 1.17) and absent metastasis at diagnosis (HR 4.9, 95% CI 1.4 to 17.2).
Predictors of disease specific survival include local disease at presentation, complete tumor resection and tumor grade, size, location and histological subtype. With adequate surgical treatment most patients who presented with primary disease and underwent complete surgical resection achieved prolonged disease specific survival.
泌尿生殖系统肉瘤较为罕见。我们描述了在25年期间单一机构的持续经验。
回顾了1977年7月至2003年7月在本机构诊断时年龄在16岁及以上、患有起源于尿路或男性生殖系统肉瘤的所有患者的记录。
131例患者的原发肿瘤部位,膀胱20例,肾脏26例,睾丸旁57例,前列腺21例,其他7例。中位随访时间为4.0年。最常见的组织学亚型为平滑肌肉瘤,占29%,脂肪肉瘤占26%。肿瘤中位大小为7cm,78%的病变为高级别。28例(21%)患者出现转移性疾病,其中位生存期为1.4年,而无转移性疾病患者的中位生存期为10.7年(p<0.0001)。疾病特异性生存率在5年和10年时分别为56%和42%,中位生存期为7.6年。单因素分析显示,疾病特异性生存的不良预后变量包括就诊时转移、肿瘤高级别、缺乏平滑肌肉瘤和脂肪肉瘤组织学亚型、前列腺肉瘤、肿瘤大、手术切除不完全和手术切缘阳性。患者性别、年龄和手术切缘状态不是显著的预测因素。多因素分析显示,2个变量仍然是疾病特异性生存的显著预测因素,包括肿瘤大小(HR 1.1,95%CI 1.02至1.17)和诊断时无转移(HR 4.9,95%CI 1.4至17.2)。
疾病特异性生存的预测因素包括就诊时的局部疾病、肿瘤完全切除以及肿瘤分级、大小、位置和组织学亚型。通过充分的手术治疗,大多数患有原发性疾病并接受完全手术切除的患者实现了延长的疾病特异性生存。