histiocytic and dendritic cells neoplasms: clinical behaviour and response to chemotherapy elena...
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Histiocytic and dendritic cells neoplasms:
clinical behaviour and response to chemotherapy
Elena PalassiniIstituto Nazionale TumoriMilano
Stacchiotti S, Gentile A, Grosso F, Palassini E, Collini P, Bertulli R, Dileo P, Messina A, Morosi C, Gronchi A, Dei Tos
AP, Casali PG
Background (1)
Histiocytic sarcoma (HS) Langherans cell sarcoma (LCS) Interdigitating dendritic cell sarcoma (IDCS) Follicular dendritic cell sarcoma (FDCS) Fibroblastic reticular cell tumor (FRCT) Unclassifiable dendritic cell tumor (UDCT)
Group of heterogeneous neoplasms showing differentiation toward accessory cells of immune system
Background (2)
Surgery is felt to be the mainstay of treatment in localized presentations
The role of radiotherapy is undetermined Evidence of chemoresponsiveness to different regimens
(including CHOP, ABVD, DHAP, IE) is based on case reports and small case series, but the role of chemotherapy remains still undefined
Patients and methods (1)
period 2002-2008 # pts 24 M/F 16/8 age: mean (range) yrs 45 (30-79) site: nodal/extranodal 12/12 stage at presentation:
Iocalized/locally advanced/metastatic 18/1/5
g
UDC: 3 pts
Patients and methods (2)
LCS: 1HS: 5 IDCS: 4 FDCS: 11 UDCT: 3
Grade: low/high 8/16
Patients and methods (3)
treatment (24):
surgery/radiotherapy/chemotherapy 19/10/12
Results (1)
# pts 12 adjuvant/neoadjuvant/palliative 1/3/8 HS/LCS/IDCS/FDCS/UDCT 2/1/2/6/1
Front-line doxorubicin + cisplatin
BIOPSY
wks -4 0 3 6
9
12 15 18
Pt 1, IDCS, localized disease
Dox+
DDP
Dox+
DDP
Dox+
DDP
Dox+
DDP
Dox+
DDP
SURGERY
RT
Treatment plan
9
Baseline Dox + DDP x1
Baseline Dox + DDP x3
HE
S-100
Results (1)
evaluable for response 11 (HS/LCS/IDCS/FDCS/UDCS) (2/1/2/5/1)
PR RECIST (HS/LCS/IDCS/FDCS/UDCS) 8 (2/1/1/3/1)
SD RECIST ( HS/LCS/IDCS/FDCS/UDCS) 2 (0/0/1/1/0)
PD RECIST ( HS/LCS/IDCS/FDCS/UDCS) 1 (0/0/0/1/0)
■ consolidation with HD chemotherapy
and PBPC support 2
Front-line doxorubicin + cisplatin: activity
Baseline Dox + DDP x6
Pt 2, HS
Baseline Dox + DDP x6
Pt 3, FDCS
Baseline Dox + DDP x4
Dox + DDP x6HD IFX x1
Results (2)Dox + DDP: PFS in the palliative setting (8 pts)
PFS median 8 mos
PFS <6 mos 3 pts
FFP @30 mos 2 pts
Results (3)
etoposide + ifosfamide 3
PR 2
PD 1 gemcitabine + vinorelbine 1
PR 1 gemcitabine 1
PR 1 high dose ifosfamide 2
PD 2
Further-line chemotherapy
Conclusions
Small series, though among the largest in literature Doxorubicin + cisplatin is active in histiocitic and dendritic
cell neoplasms (RR 73%) All subtypes seem to be sensitive Response can be short-lasting (PFS <6 mos) and tumor
behaviour very aggressive Long PFS (PFS >30 mos) was observed in 3 metastatic
patients out of 8 Consolidation with high-dose chemotherapy is worth
testing Other drugs may be active (gemcitabine, gemcitabine and
vinorelbine, ifosfamide and etoposide)
