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Review
. 2010 Oct 11:5:67.
doi: 10.1186/1746-1596-5-67.

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

Haiwei Wang  1 , Zhansan Su, Zhongliang Hu, Jifang Wen, Baoan Liu
Affiliations
Review

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

Haiwei Wang et al. Diagn Pathol. .

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.

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Figures

Figure 1
Figure 1
Case 1 FDCS of the tonsil, the spindle cells are arranged in a storiform and whorled patterns. (H&E stain).
Figure 2
Figure 2
Case 1 FDCS of the tonsil, individual cells possess characteristic features, including a delicate nuclear membrane, vesicular nuclei, small but distinct nucleoli and indistinct cell borders. (H&E stain).
Figure 3
Figure 3
Case 3 FDCS of the liver, the giant cells and mitotic figures are identified occasionally. (H&E stain).
Figure 4
Figure 4
Case 4 FDCS of the lymph node, the spindle cells are dispersed in distribution, scattered by a prominent lymphoplasmacytic infiltrate (H&E stain).
Figure 5
Figure 5
Case 1 FDCS of the tonsil, the tumor cells are immunoreactive with CD21 strongly and diffusely.
Figure 6
Figure 6
Case 4 FDCS of lymph node, the tumor cells were positive for CD35 strongly and diffusely.
See this image and copyright information in PMC

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