Wednesday, September 19th, 2018


A full text version of this article is available.
To access article obtain online access here or login
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.
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.
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
  Acrobat Reader 7.0 is recommended to properly view and print the article.
Reader can be downloaded from