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2015 ; 51
(16
): 2413-22
Nephropedia Template TP
gab.com Text
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English Wikipedia
Impact of surgery, radiation and systemic therapy on the outcomes of patients
with dendritic cell and histiocytic sarcomas
#MMPMID26298731
Gounder M
; Desai V
; Kuk D
; Agaram N
; Arcila M
; Durham B
; Keohan ML
; Dickson MA
; D'Angelo SP
; Shukla N
; Moskowitz C
; Noy A
; Maki RG
; Herrera DA
; Sanchez A
; Krishnan A
; Pourmoussa A
; Qin LX
; Tap WD
Eur J Cancer
2015[Nov]; 51
(16
): 2413-22
PMID26298731
show ga
BACKGROUND: Neoplasms of histiocytic and dendritic cell origin, including
follicular dendritic cell sarcoma (FDCS), histiocytic sarcoma (HS) and
interdigitating dendritic cell sarcoma (IDCS), are extremely rare, and data on
their natural history and treatment outcomes are sparse. We evaluated the impact
of surgery, radiation and systemic therapies on overall survival (OS). METHODS:
We conducted a retrospective chart review of patients with FDCS, IDCS and HS
treated at Memorial Sloan Kettering Cancer Center between 1995 and 2014. RESULTS:
We identified 31, 15 and 7 patients with FDCS, HS and IDCS, respectively. Median
age was 48.7, 42.3 and 58.8years for FDCS, HS and IDCS, respectively. Only a
slight disparity in gender distribution existed for FDCS and HS; however, IDCS
predominantly affected males (6:1). The most common sites of presentation were
abdomen and pelvis (42%), extremities (33%) and head and neck (57%) for FDCS, HS
and IDCS, respectively. At diagnosis, 74%, 40% and 86% of patients presented with
localised disease in FDCS, HS and IDCS, respectively. Patients with localised
disease had significantly improved OS than those with metastatic disease in FDCS
(P=0.04) and IDCS (P=0.014) but not in HS (P=0.95). In FDCS and HS, adjuvant or
neo-adjuvant therapy was not associated with improved OS compared with
observation. In IDCS, surgery alone provided a 5-year overall survival rate of
71%. CONCLUSIONS: Adjuvant or neo-adjuvant treatment in FDCS and HS did not
affect OS. Patients with IDCS had an excellent outcome with surgery. In the
metastatic setting, chemotherapy and small molecule inhibitors may provide
benefit.