Table 3 Retrospective trials included in the analysis showing the name of the author, year of publication, number of patients enrolled in the trial, the intervention that was tested and the outcome

Mitchell[34]

22 resectable dediff CS

Doxorubicin based chemotherapy + surgery

1/5 > 90% necrosis after preoperative chemo

5 preoperative chemotherapy and 6 postoperative, 11 no chemotherapy

5 year OS with chemotherapy 36%, without 0%

Dickey[35]

42 local or advanced dediff CS

15 Surgery

5 year OS and median OS: with surgery 11.8% and 6.4 months

22 surgery and chemotherapy 5 other

With surgery and chemotherapy 4% and 8.4 months

Staals[36]

102 local or advanced dediff central CS

68 Surgery

Median survival 18 vs 23 months (not significant)

25 surgery + chemotherapy

9 palliative care

Cesari[7]

24 local or advanced mesenchymal CS

24 surgery of which:

10 year OS:

5 + RT

Surgical remission 27%

12 + chemotherapy

Non-surgical remission 0%

Complete surgical remission 10 year DFS:

With chemotherapy 76%, without 17%

Grimer[37]

266 local and 71 advanced dediff CS

207 Surgery

Local disease

90 chemotherapy

5 year OS:

40 surgery + chemotherapy

With chemotherapy 33%, without 25% (p = 0.11)

Advanced disease

Median OS: With chemotherapy 7 months, without 3 months (not significant)

Dantonello[38]

14 local mesenchymal CS

12 Surgery + chemotherapy

10 year OS 64%

2 surgery + RT

Bernstein-Molho[39]

9 advanced CS

Sirolimus + cyclophosphamide

OR 11%

SD 56%

PFS 15 months

Italiano[5]

98 advanced CS

Doxorubicin based chemotherapy

OR 14%

SD 31%

PFS 5.3 months

OS 19 months