Table 4 Recent published chordoma retrospective and prospective studies with multidisciplinary approach.
From: Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients
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%. |