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| Number of patients | Intent of chemotherapy | Agents used | Response | Time to progression | Survival |
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McAfee et al. [14] prospective | 9 patients received CTx local to metastatic disease | Adjuvant | Carboplatin, cisplatin, VP-16, etoposide | NDA | 5-year local control of all cases: 94%; 5-year locoregional control of all cases: 80% | 5-year survival of all cases: 37%; 5-year DSS of all cases: 52% |
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Poulsen et al. [15] prospective | local or locoregional disease | Adjuvant (72%); therapeutic (28%) | CTx combined with RTx; etoposide, carboplatin | NDA | NDA | 3-year OS 76%; 3-year DFS 65% |
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George et al. [16] retrospective | metastatic disease | Adjuvant | Cyclophosphamide, doxorubicin, vincristin | CR: 4-5 months | 4 months | NDA |
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Redmond III et al. [17] retrospective | metastatic disease | Adjuvant | Cisplatin + etoposide or Cisplatin + etoposide + cyclophosphamide or Cyclophosphamide + doxorubicin + vincristin | CR in 5 patients | CR for a median of 3,5 months | median OS of 6,5 months |
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Azagury et al. [18] retrospective | metastatic disease | Adjuvant | First line: VP-16 + cisplatin + doxorubicin + bleomycin — Second line: RTx combined with methotrexate + cyclophosphamide + VP-16 | CR after 6 cycles — CR after 4 months | 15 months until relapse — NDA | NDA |
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Bajetta et al. [19] prospective | NET (1 MCC among all cases) locally advanced or metastatic disease | Adjuvant | 5-Fluorouracil + dacarbazine + epirubicin | CR in 2 patients (in 1 patient with MCC); PR in 7 patients | CR of MCC for 21+ months; median duration of response of all cases was 10 months (range, 5+ to 24+) | NDA |
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Eng et al. [20] retrospective | ; 43 patients received RT and/or combined CTx local to metastatic disease | Adjuvant | 19% received CTx without RTx most common regimen: VP-16 + cisplatinum | NDA; of all cases 12% had persistent disease, 40% had recurrent disease | Of all cases median time to recurrence was 8 months | NDA |
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Eng et al. [21] retrospective | 9 patients received CTx metastatic disease | Adjuvant | +/− RTx most common regimen: carboplatin + etoposide + vinblastin | NDA | Overall median time to recurrence was 9 months | OS rate of all cases: 37% |
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Veness et al. [22] retrospective | 7 patients received CTx locoregional disease | Adjuvant | most common: based on platinum | NDA; 55% of all cases experienced a relapse | median time to relapse was 3–7 months | 5-year OS 47%; DFS rate 25% |
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Voog et al. [23] retrospective | advanced or metastatic disease | Adjuvant | different regimens; NDA | ORR to first line CTx: 64% | NDA | 3-year OS was 17% (metastasis) and 35% (locally advanced) |
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Allen et al. [24] retrospective | 28 patients received CTx local or regional disease | Adjuvant | most common regimen: carboplatin + etoposide | NDA; disease recurred in 102 patients | of all cases median time to recurrence was 9 months (range, 2 to 70 months) | 5-year DFS of all cases: 48%; 5-year DSS of patients receiving CTx: 28% |
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Wobser et al. [25] retrospective | metastatic disease | Adjuvant | +RTx liposomal doxorubicin | PR in 4 patients | an average of 2 months until progression | Survival time: range 3 to 20 months |
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Schlaak et al. [26] retrospective | metastatic disease | Adjuvant | Etoposide | CR in 3 patients | 2 of CR lasted for 16 and 36 months | NDA |
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Tai et al. [27] retrospective | locoregional and metastatic disease | Adjuvant | most common regimens: Cyclophosphamide + doxorubicin (or epirubicin) + vincristin +/− prednisone — Etoposide + cisplatin (or carboplatin) | ORR 75,7% (35,1% CR, 35,1% PR, 5,4% minor responses) — ORR 60% (36% CR, 24% PR) | Median response duration of patients receiving CTx: 1–12 months | Median OS of all cases: 21,5 months (range, 1 to 118 months); 2-year OS of all cases: 36%; 5-year OS of all cases: 17% |
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