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Title:
Extranodal Follicular Dendritic Cell Sarcomas: Inflammatory Pseudotumor-like Variant with Increased Frequency of a Concurrent Secondary Cancer
Authors:  Shien-Tung Pan, M.D., Tiing Foong Siow, M.D., Khin Than Win, M.D., Tsung-Heng Tsai, M.D., Chih-Yuan Cheng, M.D., and Shih-Sung Chuang, M.D.
  Objective: To analyze the clinicopathological features of extranodal follicular dendritic cell (FDC) sarcomas in Taiwan.
Study Design:
Extranodal FDC sarcoma is rare and can be classified into either conventional type or inflammatory pseudotumor (IPT)–like variant. Nearly all gastrointestinal FDC tumors are of the conventional type and Epstein-Barr virus (EBV) unrelated, while the IPT-like variant occurs almost exclusively in the liver and spleen and is consistently associated with EBV. We report 3 new cases of extranodal FDC sarcoma in Taiwan and review their features along with 3 previously published cases.
Results:
The cases included 2 males and 4 females with a median age of 73 (range, 37–78). Two cases were of the conventional type, and 4, IPT-like variant. Immunohistochemically, all tumors except 1 case expressed at least 1 FDC marker. EBV was detected in 3 of the 4 cases of IPT-like variant by in situ hybridization. The single case with EBV-negative IPT-like variant was considered to be secondary hepatic involvement by an omental/mesenteric FDC sarcoma. Two patients had concurrent cancers in other organs. The clinical course was indolent in most cases after operation, although follow-up was either unavailable or short in some patients.
Conclusion:
We found that FDC sarcomas in Taiwan occur more commonly in elder females, are frequently of the IPT-like variant, with an indolent course, and with a high proportion of patients having a concurrent cancer in a second organ.
Keywords:  Epstein-Barr virus, extranodal follicular dendritic cell sarcoma, follicular dendritic cell, follicular dendritic cell sarcoma, inflammatory pseudotumor, Taiwan
   
   
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