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Table 3 Description of responses by histotypes and targeted therapy

From: The off-label use of targeted therapies in sarcomas: the OUTC’S program

  

TT1

n

%

CR

PR

RR (%)

TT2

n

%

CR

PR

RR (%)

Other PRs (n)

Total

278*

   

3

22

    

0

5

 

5

GIST

39

sorafenib

31

79

0

3

10

nilotinib

3

8

0

0

0

Leiomyosarcoma

36

sorafenib

22

61

0

3

14

sunitinib

9

25

0

2

22

Angiosarcoma

18

sorafenib

14

78

0

3

21

sunitinib

2

11

0

1

50

Unclassified sarcoma

15

sunitinib

8

53

0

1

13

sorafenib

5

33

0

0

0

Chordoma

15

imatinib

8

53

0

0

0

sunitinib

3

20

0

0

0

erlotinib (1)

Osteosarcoma

15

sirolimus

10

67

0

0

0

sorafenib

3

20

0

0

0

Synovialosarcoma

15

sunitinib

8

53

0

4

50

sorafenib

3

20

0

0

0

pazopanib (1)

Ewing / PNET

14

sunitinib

8

57

1

0

13

sorafenib

2

14

0

0

0

sirolimus + cyclop (1)

Chondrosarcoma

12

sirolimus

5

42

0

0

0

sorafenib

3

25

0

0

0

Uterine leiomyosarcoma

12

sorafenib

6

50

0

1

17

sunitinib

4

33

0

0

0

Liposarcoma

12

sorafenib

7

58

0

0

0

sunitinib

3

25

0

0

0

Solitary fibrous tumor

10

sorafenib

3

30

0

0

0

sunitinib

2

20

0

0

0

beva + TMZ (1)

Epithelioid sarcoma

9

sorafenib

2

22

0

1

50

sunitinib

2

22

0

0

0

beva + pacli (1)

MPNST

8

sorafenib

4

50

0

0

0

sunitinib

2

25

0

1

50

ASPS

8

sorafenib

5

63

0

2

40

sunitinib

3

38

0

0

0

DSRCT

6

sorafenib

3

50

0

0

0

sunitinib

3

50

0

0

0

AF

6

imatinib

4

67

0

0

0

sorafenib

1

17

0

1

100

DFSP

5

imatinib

4

80

1

2

75

sunitinib

1

20

0

0

0

PEComa

4

temsirolimus

2

50

1

1

100

sirolimus

1

25

0

0

0

Rhabdomyosarcoma

3

sunitinib

2

67

0

0

0

sorafenib

1

33

0

0

0

KS

1

everolimus

1

100

0

1

100

NA

Low grade ESS

1

sorafenib

1

100

0

0

0

NA

Phyllode tumor

1

sunitinib

1

100

0

0

0

NA

Other (including benign tumors)

13

ND

 

ND

  1. Complete and partial responses are indicated in bold.
  2. *represents the total number of treatment lines (some patients had several lines of targeted therapy).
  3. TT1 is the most frequent targeted therapy, TT2 is the second most frequent targeted therapy.
  4. AF: aggressive fibromatosis.
  5. ASPS: alveolar soft parts sarcoma.
  6. beva: bevacizumab.
  7. CR: complete response.
  8. cyclop: cyclophosphamide.
  9. DFSP: dermatofibrosarcoma protuberans.
  10. DSRCT: desmoplastic small round cell tumor.
  11. ESS: endometrial stromal sarcoma.
  12. GIST: gastro-intestinal stromal tumor.
  13. KS: kaposi sarcoma.
  14. MPNST: malignant peripheral nerve sheath tumor.
  15. NA: not applicable.
  16. ND: not described.
  17. pacli: paclitaxel.
  18. PR: partial response.
  19. RR: response rate.
  20. TMZ: temozolomide.
  21. TT: targeted therapy.