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Histiocytic and dendritic cells neoplasms : clinical behaviour and response to chemotherapy
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Histiocytic and dendritic cells neoplasms : clinical behaviour and response to chemotherapy

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  1. Histiocytic and dendritic cells neoplasms: clinical behaviour and response to chemotherapy 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 Elena Palassini Istituto Nazionale Tumori Milano

  2. Background (1) • Group of heterogeneous neoplasms showing differentiation toward accessory cells of immune system 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)

  3. 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

  4. 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

  5. Patients and methods (2) UDC: 3 pts UDCT: 3 HS: 5 LCS: 1 IDCS: 4 FDCS: 11 • Grade: low/high 8/16 g

  6. Patients and methods (3) • treatment (24): surgery/radiotherapy/chemotherapy 19/10/12

  7. Results (1) Front-line doxorubicin + cisplatin • # pts 12 • adjuvant/neoadjuvant/palliative 1/3/8 • HS/LCS/IDCS/FDCS/UDCT 2/1/2/6/1

  8. Pt 1, IDCS, localized disease Treatment plan 9 wks -4 0 3 6 9 12 15 18 RT Dox + DDP Dox + DDP Dox + DDP Dox + DDP Dox + DDP BIOPSY SURGERY

  9. Baseline Dox + DDP x1

  10. HE S-100 Baseline Dox + DDP x3

  11. Results (1) Front-line doxorubicin + cisplatin: activity • 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

  12. Pt 2, HS Baseline Dox + DDP x6

  13. Baseline Dox + DDP x6

  14. Pt 3, FDCS Baseline Dox + DDP x4 HD IFX x1 Dox + DDP x6

  15. 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

  16. Results (3) Further-line chemotherapy • etoposide + ifosfamide 3 PR 2 PD 1 • gemcitabine + vinorelbine 1 PR 1 • gemcitabine 1 PR 1 • high dose ifosfamide 2 PD 2

  17. 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)

  18. elena.palassini@istitutotumori.mi.it silvia.stacchiotti@istitutotumori.mi.it paolo.casali@istitutotumori.mi.it