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)

elena.palassini@istitutotumori.mi.it

silvia.stacchiotti@istitutotumori.mi.it

paolo.casali@istitutotumori.mi.it