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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%.
  1. 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.