Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma

医学 内科学 肿瘤科 恶性肿瘤 吉西他滨 滤泡树突状细胞 放射治疗 疾病 胃肠病学 化疗 T细胞 免疫学 抗原提呈细胞 免疫系统
作者
Preetesh Jain,Sarah A. Milgrom,Keyur P. Patel,Loretta J. Nastoupil,Luis Fayad,Michael Wang,Chelsea C. Pinnix,Bouthaina S. Dabaja,Grace L. Smith,Jun Yu,Shimin Hu,Claudio Rodríguez Ramos,Rashmi Kanagal‐Shamanna,L. Jeffrey Medeiros,Yasuhiro Oki,Nathan Fowler
出处
期刊:British Journal of Haematology [Wiley]
卷期号:178 (3): 403-412 被引量:61
标识
DOI:10.1111/bjh.14672
摘要

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 (FDCS). Six patients also had Castleman disease, 9 had another malignancy and 13 had an autoimmune disease. Fifty-four per cent of patients presented with localized disease and 46% with systemic involvement. The median progression-free (PFS) and overall survival (OS) following frontline therapy was 21 and 50 months, respectively. Survival outcomes were significantly inferior in patients with extranodal, bulky or intra-abdominal disease at presentation. Stage was not associated with survival. Management approaches were heterogeneous. Patients who underwent an upfront gross total resection (GTR) experienced better PFS and OS (both P < 0·0001). In patients who underwent a GTR, consolidative radiotherapy was associated with improved local control (P = 0·03), PFS (P = 0·04) and OS (P = 0·05). 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 FDCS and identify potential novel therapeutic targets.
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