PDF(Pubmed)
Abstract：
Synovial sarcoma (SS) is a rare soft-tissue tumor characterized by a monomorphic blue spindle cell histology and variable epithelial differentiation. Morphologically, SSs may be confused with other sarcomas. Systemic treatment is more effective for patients with high-risk SSs, patients with advanced disease, and younger patients. However, further studies are required to find new prognostic biomarkers. Herein, we describe the morphological, molecular, and clinical findings, using a wide immunohistochemical panel, of a series of SS cases. We studied 52 cases confirmed as SSs by morphological diagnosis and/or molecular studies. Clinical data (gender, age, tumor size, tumor location, resection margins, adjuvant treatment, recurrences, metastasis, and survival) were also retrieved for each patient. All the available H&E slides were examined by four pathologists. Three tissue microarrays (TMAs) were constructed for each of the tumors, and a wide immunohistochemical panel was performed. For time-to-event variables, survival analysis was performed using Kaplan-Meier curves and log-rank testing, or Cox regression. Statistical significance was considered at p < 0.05. The mean age of our patients was 40.33, and the median was 40.5 years. We found a predominance of males versus females (1.7:1). The most frequent morphological subtype was monophasic. TRPS1, SS18-SSX, and SSX-C-terminus were positive in 96% of cases. GLI1 expression was strong in six and focal (cytoplasmic) in twenty patients. Moreover, BCOR was expressed in more than half of SSs. Positive expression of both proteins, BCOR and GLI1, was correlated with a worse prognosis. Multivariate analysis was also performed, but only BCOR expression appeared to be significant. The combination of GLI1 and BCOR antibodies can be used to group SSs into three risk groups (low, intermediate, and high risk). We hypothesize that these findings could identify which patients would benefit from receiving adjuvant treatment and which would not. Moreover, these markers could represent therapeutic targets in advanced stages. However, further, larger series of SSs and molecular studies are necessary to corroborate our present findings.
摘要：
滑膜肉瘤（SS）是一种罕见的软组织肿瘤，其特征是单形的蓝色梭形细胞组织学和可变的上皮分化。形态学上,SS可能与其他肉瘤混淆。系统治疗对高危SSs患者更有效，晚期疾病患者，年轻患者。然而,需要进一步的研究来寻找新的预后生物标志物.在这里，我们描述了形态学，分子，和临床发现，使用广泛的免疫组织化学面板，一系列党卫军案件。我们研究了52例经形态学诊断和/或分子研究证实为SS的病例。临床数据（性别，年龄,肿瘤大小,肿瘤位置,切除边缘,辅助治疗,复发，转移,和生存率）也为每位患者检索。所有可用的H&E载玻片由四名病理学家检查。为每个肿瘤构建了三个组织微阵列（TMA），并进行了广泛的免疫组织化学面板。对于时间到事件变量，使用Kaplan-Meier曲线和对数秩检验进行生存分析，或Cox回归。在p<0.05时考虑统计学显著性。我们患者的平均年龄为40.33岁，中位数为40.5岁。我们发现男性与女性的优势（1.7：1）。最常见的形态亚型是单相。TRPS1,SS18-SSX,在96%的病例中，SSX-C-末端呈阳性。GLI1在6例患者中表达强烈，在20例患者中出现局灶性（细胞质）。此外，BCOR在半数以上的SS中表达。两种蛋白的阳性表达，BCOR和GLI1与不良预后相关。还进行了多变量分析，但只有BCOR表达似乎是显著的。GLI1和BCOR抗体的组合可用于将SS分为三个风险组（低，中间,和高风险)。我们假设这些发现可以确定哪些患者会从接受辅助治疗中受益，哪些不会。此外，这些标志物可以代表晚期的治疗靶点.然而,进一步,更多系列的SS和分子研究是必要的，以证实我们目前的发现。
