TY - JOUR
T1 - Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma
AU - Jain, Preetesh
AU - Milgrom, Sarah A.
AU - Patel, Keyur P.
AU - Nastoupil, Loretta
AU - Fayad, Luis
AU - Wang, Michael
AU - Pinnix, Chelsea C.
AU - Dabaja, Bouthaina S.
AU - Smith, Grace L.
AU - Yu, Jun
AU - Hu, Shimin
AU - Bueso Ramos, Carlos E.
AU - Kanagal-Shamanna, Rashmi
AU - Medeiros, L. Jeffrey
AU - Oki, Yasuhiro
AU - Fowler, Nathan
N1 - Publisher Copyright:
© 2017 John Wiley & Sons Ltd
PY - 2017/8
Y1 - 2017/8
N2 - 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.
AB - 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.
KW - dendritic cell sarcoma
KW - follicular dendritic cell sarcoma
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U2 - 10.1111/bjh.14672
DO - 10.1111/bjh.14672
M3 - Article
C2 - 28382648
AN - SCOPUS:85017342073
SN - 0007-1048
VL - 178
SP - 403
EP - 412
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 3
ER -