{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T15:30:45Z","timestamp":1781796645601,"version":"3.54.5"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,4,6]],"date-time":"2017-04-06T00:00:00Z","timestamp":1491436800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Dendritic cell sarcomas are rare tumours of antigen presenting cells. Data regarding their biology, management and outcomes are sparse. We analysed 66 patients with follicular dendritic cell sarcoma (<jats:styled-content style=\"fixed-case\">FDCS<\/jats:styled-content>). Six patients also had Castleman disease, 9 had another malignancy and 13 had an autoimmune disease. Fifty\u2010four per cent of patients presented with localized disease and 46% with systemic involvement. The median progression\u2010free (<jats:styled-content style=\"fixed-case\">PFS<\/jats:styled-content>) and overall survival (<jats:styled-content style=\"fixed-case\">OS<\/jats:styled-content>) following frontline therapy was 21 and 50\u00a0months, respectively. Survival outcomes were significantly inferior in patients with extranodal, bulky or intra\u2010abdominal disease at presentation. Stage was not associated with survival. Management approaches were heterogeneous. Patients who underwent an upfront gross total resection (<jats:styled-content style=\"fixed-case\">GTR<\/jats:styled-content>) experienced better <jats:styled-content style=\"fixed-case\">PFS<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">OS<\/jats:styled-content> (both <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b70001). In patients who underwent a <jats:styled-content style=\"fixed-case\">GTR<\/jats:styled-content>, consolidative radiotherapy was associated with improved local control (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b703), <jats:styled-content style=\"fixed-case\">PFS<\/jats:styled-content> (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b704) and <jats:styled-content style=\"fixed-case\">OS<\/jats:styled-content> (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b705). In patients with measureable disease, gemcitabine with a taxane yielded an overall response rate of 80%. The pattern of relapse was predominantly locoregional. Salvage rates after recurrence were poor. Studies are underway at our institution to define the genomic profile in <jats:styled-content style=\"fixed-case\">FDCS<\/jats:styled-content> and identify potential novel therapeutic targets.<\/jats:p>","DOI":"10.1111\/bjh.14672","type":"journal-article","created":{"date-parts":[[2017,4,6]],"date-time":"2017-04-06T07:14:47Z","timestamp":1491462887000},"page":"403-412","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":77,"title":["Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma"],"prefix":"10.1111","volume":"178","author":[{"given":"Preetesh","family":"Jain","sequence":"first","affiliation":[{"name":"Department of Internal Medicine University of Texas Medical School at Houston Houston TX USA"},{"name":"Department of Lymphoma and Myeloma The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah A.","family":"Milgrom","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keyur P.","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loretta","family":"Nastoupil","sequence":"additional","affiliation":[{"name":"Department of Lymphoma and Myeloma The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis","family":"Fayad","sequence":"additional","affiliation":[{"name":"Department of Lymphoma and Myeloma The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Lymphoma and Myeloma The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chelsea C.","family":"Pinnix","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bouthaina S.","family":"Dabaja","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grace L.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Biostatistics The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shimin","family":"Hu","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos E.","family":"Bueso Ramos","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rashmi","family":"Kanagal\u2010Shamanna","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center Houston TX USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. 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