Email updates

Keep up to date with the latest news and content from Diagnostic Pathology and BioMed Central.

Open Access Review

Follicular dendritic cell sarcoma: a report of six cases and a review of the Chinese literature

Haiwei Wang1, Zhansan Su2, Zhongliang Hu1, Jifang Wen1 and Baoan Liu1*

Author affiliations

1 Department of Pathology, Xiangya Basic Medical School, Central South University, Changsha, Hunan Province, 410013, China

2 Department of Pathology, Xiangya Third Hospital, Central South University, Changsha, Hunan Province, 410013, China

For all author emails, please log on.

Citation and License

Diagnostic Pathology 2010, 5:67  doi:10.1186/1746-1596-5-67

Published: 11 October 2010

Abstract

Goals

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.

Methods

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.

Results

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.

Conclusions

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.