Department of Pathology, Xiangya Basic Medical School, Central South University, Changsha, Hunan Province, 410013, China.
Diagn Pathol. 2010 Oct 11;5:67. doi: 10.1186/1746-1596-5-67.
The main purpose of this study is to broaden the clinicopathological spectrum and increase recognition of follicular dendritic cell sarcoma (FDCS) through analysis of the clinical and pathological features of 50 cases.
The clinicopathological features of total 50 cases of FDCS were analyzed including a review of 44 cases reported in Chinese literature before October 2009 and six original cases from the pathology files conducted by the authors.
The youngest patient came under observation in this study is only seven years old. Including the cases contributed by the authors, our literary review indicated that male dominated the tumor cases (M: F = 3: 2). 28 cases (56%) present with this disease in extranodal sites. Tumor cells demonstrated positive staining for the follicular dendritic cell markers CD21 (47/49), CD35 (43/45), CD23 (20/23) and CD68 (23/25). In situ hybridization for Epstein-Barr virus-encoded RNA was performed in 10 cases. Nevertheless, EBV expression was absent in all these cases. The follow-up analysis of all cases shows that 26 (81.2%) patients were alive and disease free; 6 (18.8%) patients were alive with recurrent disease or metastasis; and nobody had died of this disease at the time of last follow-up.
The diagnosis of the FDCS is based on the findings of morphology and immunohistochemistry. The FDCS occurred in China should be viewed and treated as a low-grade sarcoma, and the role of the EBV in the pathogenesis of this tumor is still uncertain. There is a possibility that the tumor might be racial or geographic correlated, because most cases were reported from Eastern Asia area; it's particular the case of the liver or spleen tumor.
本研究的主要目的是通过分析 50 例滤泡树突状细胞肉瘤（FDCS）的临床和病理特征，拓宽其临床病理谱并提高对其的认识。
分析了 50 例 FDCS 的临床病理特征，包括对 2009 年 10 月前中国文献中报道的 44 例和作者病理档案中的 6 例进行了复习。
本研究中观察到的最小患者年仅 7 岁。包括作者提供的病例在内，我们的文献回顾表明，肿瘤病例以男性为主（M：F=3：2）。28 例（56%）发生于结外部位。肿瘤细胞对滤泡树突状细胞标志物 CD21（47/49）、CD35（43/45）、CD23（20/23）和 CD68（23/25）呈阳性染色。对 10 例进行了 EBV 编码 RNA 的原位杂交，但所有病例均未见 EBV 表达。对所有病例的随访分析显示，26 例（81.2%）患者存活且无疾病；6 例（18.8%）患者存活但有复发或转移；截至最后随访时，无患者死于该病。
FDCS 的诊断基于形态学和免疫组织化学检查。在中国发生的 FDCS 应视为低度肉瘤，并不能确定 EBV 在该肿瘤发病机制中的作用。由于大多数病例来自东亚地区，尤其是肝脏或脾脏肿瘤，因此肿瘤可能与种族或地理位置有关。
