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| follicular dendritic cell sarcoma ： ウィキペディア英語版 |
follicular dendritic cell sarcoma
Follicular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm. While the existence of FDC tumors was predicted by Lennert in 1978, the tumor wasn’t fully recognized as its own cancer until 1986 after characterization by Monda et al. It accounts for only 0.4% of soft tissue sarcomas, but has significant recurrent and metastatic potential and is considered an intermediate grade malignancy. The major hurdle in treating FDCS has been misdiagnosis. It is a newly characterized cancer, and because of its similarities in presentation and markers to lymphoma, both Hodgkin and Non-Hodgkin subtypes, diagnosis of FDCS can be difficult. With recent advancements in cancer biology better diagnostic assays and chemotherapeutic agents have been made to more accurately diagnose and treat FDCS.
==Symptoms and presentation==
Follicular dendritic cells are localized in germinal centers of lymphoid follicles and have an integral role in regulation of the germinal center reaction and present antigens to B cells. Most cases of FDCS develop in the lymph nodes, but about 30% develop in extranodal sites. In 1998 the largest study on the disease was a retrospective review with fifty-one patients. Of these fifty-one patients, no conclusive pattern was found in regard to age, sex, race or presentation. The median patient age was 41 (range 14–76), and while most cases presented with cervical and axillary lymphadenopathy, 17 presented in extranodal sites including the liver, spleen, bowel and pancrease.〔 With such a range of patient histories no definitive cause has been linked to FDCS. There has, however, been some evidence that previous exposure to the Epstein Barr Virus (EBV) or diagnosis of Castleman's disease can increase the risk of developing FDCS—medical literature in 2000 reported approximately 12% of all cases of FDC tumors are associated with EBV, with variance in different organs, but the role of EBV remains unclear in FDC tumor pathogenesis; and EBV does not appear to play a role in the transformation process of Castleman's disease to FDC sarcoma because all cases the report found associated with Castleman's disease were EBV negative.〔Chen, Tse-Ching, Kuo, Tseng-tong, and Ng, Kwai-Fong, (Follicular Dendritic Cell Tumor of the Liver: A Clinicopathologic and Epstein-Barr Virus Study of Two Cases ), ''Modern Pathology'', 14(4):354–360, 2000-9-8, nature.com. ISSN 0893-3952. Accessed 2012-5-1.〕〔Horiguchi, H., Matsui-Horiguchi, M., Sakata, H., Ichinose, M., Yamamoto, T., Fujiwara, M. and Ohse, H., (Inflammatory pseudotumor-like follicular dendritic cell tumor of the spleen ), ''Pathology International'', 54:124–131, 2004-1-19, ncbi.nlm.nih.gov. PMID 14720144. Accessed 2012-5-1.〕
Symptoms of FDCS vary, and are largely dependent on the part of the body the tumor develops. The most common symptom is painless swelling in lymph nodes. This symptom alone, however, is nonconclusive as it is associated with many other diseases including the common cold. Other symptoms include cough, sore throat, difficulty swallowing, weight loss and tiredness. In cases that present in extranodal sites outside of the head and neck region, organ specific symptoms are observed.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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