Pathology Department, Hospital Universitari i Politècnic La Fe of Valencia, 46026 Valencia, Spain.
Pathology Department, University of Valencia, 46010 Valencia, Spain.
Int J Mol Sci. 2024 Jul 11;25(14):7615. doi: 10.3390/ijms25147615.
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 < 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.
滑膜肉瘤 (SS) 是一种罕见的软组织肿瘤，其特征为形态单一的蓝色梭形细胞组织学和可变的上皮分化。在形态学上，SS 可能与其他肉瘤混淆。全身治疗对高危 SS 患者、晚期疾病患者和年轻患者更有效。然而，需要进一步的研究来寻找新的预后生物标志物。在此，我们描述了一系列 SS 病例的形态学、分子和临床发现，并使用了广泛的免疫组织化学面板。我们研究了 52 例通过形态学诊断和/或分子研究确认为 SS 的病例。还为每位患者检索了临床数据（性别、年龄、肿瘤大小、肿瘤位置、切缘、辅助治疗、复发、转移和生存）。由四位病理学家检查所有可用的 H&E 切片。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分析。对于时间事件变量，使用 Kaplan-Meier 曲线和对数秩检验或 Cox 回归进行生存分析。统计学意义定义为 < 0.05。我们患者的平均年龄为 40.33 岁，中位数为 40.5 岁。我们发现男性多于女性（1.7:1）。最常见的形态亚型是单相。TRPS1、SS18-SSX 和 SSX-C 末端在 96%的病例中呈阳性。GLI1 在六例患者中表达强烈，在二十例患者中呈局灶性（细胞质）表达。此外，BCOR 在超过一半的 SS 中表达。BCOR 和 GLI1 两种蛋白的阳性表达与预后不良相关。还进行了多变量分析，但只有 BCOR 表达具有统计学意义。GLI1 和 BCOR 抗体的组合可将 SS 分为三组（低、中、高危）。我们假设这些发现可以确定哪些患者将受益于接受辅助治疗，哪些患者不会。此外，这些标志物可能成为晚期阶段的治疗靶点。然而，需要进一步的、更大的 SS 系列和分子研究来证实我们目前的发现。
