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      OncoTargets and Therapy (submit here)

      This international, peer-reviewed Open Access journal by Dove Medical Press focuses on the pathological basis of cancers, potential targets for therapy and treatment protocols to improve the management of cancer patients. Publishing high-quality, original research on molecular aspects of cancer, including the molecular diagnosis, since 2008. Sign up for email alerts here. 50,877 Monthly downloads/views I 4.345 Impact Factor I 7.0 CiteScore I 0.81 Source Normalized Impact per Paper (SNIP) I 0.811 Scimago Journal & Country Rank (SJR)

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      Follicular dendritic cell sarcoma: two rare cases and a brief review of the literature

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          Abstract

          Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor recognized in recent years. It accounts for only 0.4% of soft-tissue sarcomas, and its underlying causes are largely unknown. A correct diagnosis can be difficult to make. Diagnosis of FDCS depends on the combined clinical examination, histopathologic features, electron microscopic examination and confirmation with immunohistochemical studies. Here, we report two rare cases of FDCS: one case involving multiple bones, and the other involving extensive abdominal and pelvic cavities. Clinical, histopathological, and immunohistochemical aspects, therapeutic options, and a related literature review of the two cases are discussed. As the prevalence of FDCS is increasing, the details of these rare cases may highlight the importance and facilitate treatment of similar diseases.

          Most cited references23

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          A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation. A report of 4 cases.

          Four cases are described of a nonlymphomatous primary lymph node malignancy characterized by the proliferation of oval and spindle cells, occasionally multinucleated, and arranged in a nesting, swirling, and storiform pattern. The combination of light-microscopic, ultrastructural, and immunohistochemical features suggests that these tumors might be derived from dendritic reticulum cells.
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            Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series.

            Dendritic cell tumors are extremely rare and current knowledge on these tumors is limited. The characteristics of three dendritic cell sarcoma subtypes and their optimal treatment approaches are not fully clarified. We aimed to make a systematic review of the literature and enrich the current data with five new cases. Pooled analysis of 462 reported cases revealed that the tumor had no age, gender or racial predilection. Our analysis suggests that the young age, advanced stage, intraabdominal involvement and unfavorable histological features (i.e. large tumor size, absence of lymphoplasmacytic infiltration, coagulative necrosis, high mitotic count) may predict poor prognosis. Subtypes of this tumor have different clinical behaviors with interdigitating dendritic cell sarcoma being the most aggressive form. In general, surgery is the most effective treatment modality and adjuvant radiotherapy has no significant effect on overall survival of patients. The role of chemotherapy for the management of advanced disease is controversial. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.
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              Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized.

              The goal of this study was to characterize the clinicopathologic features of follicular dendritic cell sarcoma, a very uncommon neoplasm. The 17 cases were collected from the consultation and surgical pathology files of the authors, including 8 previously reported cases. The histologic and immunohistochemical features and outcome were analyzed. The patients had a median age of 40 years, with a slight female predominance. Seven patients presented with enlarged lymph nodes, and ten presented with tumor in extranodal sites. Two cases were associated with hyaline-vascular Castleman's disease. The tumors had an average greatest dimension of 6.7 cm. The most common histologic feature was a storiform or fascicular array of spindle, ovoid, or polygonal cells with oval nuclei, delicate nuclear membrane, vesicular or granular chromatin, distinct nucleoli, indistinct cell borders, and frequently fibrillary cytoplasm. There were often scattered multinucleated forms. The tumor cells sometimes formed sheets, circular whorls, follicle-like structures, trabeculae, or pseudovascular spaces. There was a sprinkling of small lymphocytes, with or without cuffing around blood vessels. The neoplastic cells were immunoreactive for CD21 (17 of 17 cases), CD35 (17 of 17 cases), desmoplakin (10 of 17 cases), epithelial membrane antigen (14 of 16 cases), S-100 protein (6 of 17 cases), and CD68 (2 of 17 cases), but not cytokeratin. Ultrastructural studies showed villous processes connected by desmosomes. Only one harbored the Epstein-Barr virus. Among 13 patients with a median follow-up of 3 years, local recurrence occurred in 6, metastasis in 6, and 3 died of disease. Follicular dendritic cell sarcoma exhibits distinctive histologic features that permit its presumptive recognition, but a firm diagnosis requires confirmation with special studies. Because it has a significant recurrent and metastatic potential (the latter risk having been previously underestimated), it should be viewed as an intermediate grade malignancy. An intraabdominal location is associated with a particularly aggressive clinical course.
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                Author and article information

                Journal
                Onco Targets Ther
                Onco Targets Ther
                OncoTargets and Therapy
                OncoTargets and therapy
                Dove Medical Press
                1178-6930
                2015
                24 July 2015
                : 8
                : 1823-1830
                Affiliations
                [1 ]Department of Oncology, Shandong Cancer Hospital & Institute, School of Medicine and Life Sciences, University of Jinan-Shandong Academy of Medical Sciences, Shandong Province, People’s Republic of China
                [2 ]Department of Pathology, Shandong Cancer Hospital & Institute, Jinan, Shandong Province, People’s Republic of China
                [3 ]Department of Oncology, Shandong Cancer Hospital & Institute, Jinan, Shandong Province, People’s Republic of China
                Author notes
                Correspondence: Jie Liu, Department of Oncology, Shandong Cancer Hospital, No 440 Jiyan Road, Huaiyin District, Jinan, Shandong Province 250117, People’s Republic of China, Tel +86 531 6762 6332, Fax +86 531 8798 4079, Email liujiesdch@ 123456126.com
                [*]

                These authors contributed equally to this work

                Article
                ott-8-1823
                10.2147/OTT.S86502
                4521670
                26244020
                2a3c98c6-7ca1-47b3-bf21-a99c04409859
                © 2015 Ma et al. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License

                The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

                History
                Categories
                Case Series

                Oncology & Radiotherapy
                fdcs,bone,abdominal cavity,pelvic cavity,diagnosis,therapy
                Oncology & Radiotherapy
                fdcs, bone, abdominal cavity, pelvic cavity, diagnosis, therapy

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