Table 4.
Recent published chordoma retrospective and prospective studies with multidisciplinary approach.
| Author (year) | No. of patients | Surgery (margins) | Radiotherapy | Chemotherapy (regimen) | Target therapy | Comments/Conclusions |
|---|---|---|---|---|---|---|
| Azzarelli A. (1988)[32] | 33 | 21 (8WM;13 IL/M) |
7 adjuvant 4 palliative on primary Pallliative on recurrences |
1 (PVB) 1 (meclorethamine) 1 (cyclophosphamide) 1 (doxorubicin + imidazolcarboximide) |
- | Short complete remission only after PVB. 5-, 10-, and 14-year OS rates of 30%, 10%, and 10%. |
| Bergh P. (2000) [20] | 39 | 39(23WM;16 IL/M) | 5 adjuvant 14 palliative |
- | - | 5-, 10-, and 20-year OS rates of 84%, 64%, and 52%. Local recurrence significantly associated with an increased risk of metastasis and tumor-related death (p < .001) |
| Baratti D. (2002) [10] | 28 | 28(11WM;13 M; 4IL) | 10 adjuvant 2 palliative |
2 (not specified) | - | 5- and 10-year OS and DFS rates of 87.8 and 48.9%, and 60.6 and 24.2%. |
| Boriani S. (2006) [8] | 48 | 48(18WM;20 IL; 10 palliative) | 28 adjuvant 23 palliative |
- | 1 (IM) | Only margin-free en-bloc resection associated with long CDF survival. No tumoral volume increase at 1 year from IM onset in 1 patient. |
| Chug R. (2005) [30] | 15 | 12 (not specified) |
13 | 15 (9-NC) | - | Advanced disease. 1 PR (7%) with 9-NC lasting at least 8 months and 14 SD. Median TTP 9.9 months. 3- and 6-month PFS rate of 47 and 33%. |
| Stacchiotti S. (2007) [15] | 55 | 50 (not specified) | 39 | 6 (not specified) | 55(IM) | Advanced disease. Clinical benefit (CR+PR+SD> 6 months) with IM: 71%. Median PFS: 9 months; 1-year PFS rate: 35%.;1-year OS rate: 80%. |
| Casali P.G. (2007) [34] | 6 | n.a | n.a | n.a | 6(IM + P post prior IM) | Advanced disease. 4/6 re-establishment of response with P added to IM. |
| Stacchiotti S. (2009) [36] | 10 | 10 (not specified) | 6 | - | 10 (IM+ sirolimus post prior IM+/-P) | Advanced disease. Clinical benefit with IM+sirolimus: 89%. 4 patients on treatment for > 12 months |
| Ferraresi V. (present series) | 25 | 22 (5 WM;17IL) | 3 adjuvant 8 palliative |
- | 17 (IM) 1 (nilotinib) 1 (IM+sirolimus) 1 (IM+P) |
5- and 10-year survival rate of 76.7 and 59.7%. 2-year local PFS rate: 53%. 5-year distant metastasis free survival: 78.3%. |
WM: wide margins; IL: intralesional margins; M: marginal margins; PVB: cisplatinum/vinblastine/bleomycin; OS: overall survival; DFS: disease-free survival; IM: imatinib mesylate; CDF: continuously disease free; 9-NC: 9-nitro-camptothecin; PR: partial response; SD: stable disease; TTP: time to progression; PFS: progression-free survival; CR: complete response; P: cisplatinum.