Department of Radiology, Oncology and Human Pathology, Oncology Unit B, Sapienza University of Rome, Italy.
BJU Int. 2012 Nov;110(10):1449-54. doi: 10.1111/j.1464-410X.2012.11105.x. Epub 2012 Mar 27.
To describe, for the first time, the clinical characteristics of primary renal synovial sarcoma (SS) and to examine the association of histological features with the expression of immunohistochemical markers.
We collated published data on all cases of primary renal SS, from its first description in 2000 to September 2011. Data on clinical and pathological characteristics were extracted and used to create a database. Disease-free survival (DFS) and overall survival (OS) rates were estimated using the Kaplan-Meier method with Rothman's 95% confidence intervals (CIs) and compared across the groups using the log-rank test. The associations between tumour extension and histological features were evaluated using the non-parametric Spearman rank test. A chi-squared test was used to assess the differences between groups.
In the overall cohort, the median OS was 48 months (95% CI, 14.1-81.9). Cox analysis showed that the risk of death at diagnosis was greatly increased in patients with metastatic disease compared with those with non-metastatic disease (hazard ratio [HR]: 343.9, 95% CI, 2.8-42,000; P= 0.017). The median DFS was 33.0 months (95% CI, 16.8-49.2), and patients who develop metastatic disease have a very poor prognosis with a median survival of 6 months (95% CI, 5.1-6.9). Microscopic features were monophasic, biphasic and poorly differentiated synovial sarcoma in 76, 16 and 8% of patients, respectively. Significant differences in expression of immunohistochemical markers or genetic mutation were found between different subtypes.
Despite its retrospective nature, this study shows that renal SS comprises different histological subtypes, which are characterized by specific immunohistochemical stains and by specific translocations. When diagnosed at metastatic stage, the prognosis was very poor compared with that for non-metastatic disease, even though one out of three patients with non-metastatic disease had disease relapse. Cooperative efforts and publication of cases with adequate follow-up are necessary to better define prognosis and therapeutic strategies for this rare disease.
首次描述原发性肾滑膜肉瘤（SS）的临床特征，并研究组织学特征与免疫组化标志物表达的相关性。
我们对 2000 年至 2011 年 9 月期间发表的所有原发性肾 SS 病例进行了文献回顾。提取临床和病理特征数据，并创建数据库。采用罗特曼 95%置信区间（CI）的 Kaplan-Meier 法估计无病生存（DFS）和总生存（OS）率，并采用对数秩检验比较各组间的差异。采用非参数 Spearman 秩检验评估肿瘤扩展与组织学特征之间的关系。采用卡方检验评估组间差异。
在整个队列中，中位 OS 为 48 个月（95%CI，14.1-81.9）。Cox 分析显示，与无转移疾病患者相比，诊断时患有转移性疾病的患者死亡风险大大增加（风险比[HR]：343.9，95%CI，2.8-42，000；P=0.017）。中位 DFS 为 33.0 个月（95%CI，16.8-49.2），发生转移性疾病的患者预后极差，中位生存时间为 6 个月（95%CI，5.1-6.9）。76%、16%和 8%的患者分别为单相、双相和低分化滑膜肉瘤。不同亚型之间的免疫组化标志物表达或基因突变存在显著差异。
尽管本研究为回顾性研究，但结果表明，肾 SS 包含不同的组织学亚型，其特征为特定的免疫组化染色和特定的易位。与非转移性疾病相比，当诊断为转移性疾病时，患者的预后非常差，尽管三分之一的非转移性疾病患者有疾病复发。为了更好地确定这种罕见疾病的预后和治疗策略，需要进行合作努力并公布具有充分随访的病例。
