Table 1: Clinical, Pathologic, Molecular Features, Management
and Outcome IFS.
|
Patient |
Sex |
Age
(months) |
Symptoms |
Site |
Size |
Mtts |
PCR ETV6-NTRK3 |
FISH
ETV6 |
Initial
surgery |
IRS
post 1st Sx |
Chemo |
2nd
surgery |
IRS
post 2nd Sx |
Event |
Status |
OS (months) |
|
1 |
M |
8.29 |
Gastrointestinal
bleeding |
Intestinal |
<5cm |
no |
Positive |
Positive |
Complete
resection |
I |
No |
Yes
|
I |
No |
CR |
25.44 |
|
2 |
F |
1.48 |
Tumor |
Limb |
>5cm |
no |
Negative* |
Negative |
Biopsy |
III |
Yes |
No |
- |
PD |
SD |
34 |
|
3 |
F |
0.66 |
Tumor
/ Bleeding |
Limb |
>5cm |
no |
Positive |
Positive |
Biopsy |
III |
Yes |
Yes |
II |
No |
SD |
33.31 |
|
4 |
F |
3.45 |
Shortness
of breath |
Trunk |
>5cm |
no |
Positive |
Positive |
Biopsy |
III |
Yes |
Yes |
I |
No |
CR |
65.8 |
|
5 |
M |
2.04 |
Tumor |
Limb |
>5cm |
no |
Positive |
Positive |
Partial
resection |
III |
Yes |
No |
- |
No |
CR |
15.97 |
|
6 |
M |
0.95 |
Tumor |
Limb |
>5cm |
no |
Negative |
Negative |
Complete
resection |
II |
No |
No |
- |
No |
CR |
71.93 |
|
7 |
M |
8.22 |
Tumor |
Limb |
<5cm |
no |
Positive |
Positive |
Complete
resection |
II |
No |
No |
- |
No |
CR |
37.84 |
|
8 |
M |
3.98 |
Tumor
/ Bleeding |
Limb |
>5cm |
no |
Positive |
Positive |
Partial
resection |
II |
Yes |
Yes |
I |
No |
CR |
87.9 |
|
9 |
M |
9.70 |
Tumor |
H&N |
<5cm |
no |
Negative |
N/A |
Biopsy |
III |
No |
No |
- |
No |
CR |
108.23 |
|
10 |
M |
11.31 |
Tumor |
Limb |
>5cm |
lung |
Positive |
Positive |
Biopsy |
III** |
Yes |
No |
- |
Death |
Death |
11.12 |
|
11 |
M |
2.27 |
Tumor |
Limb |
>5cm |
no |
Positive |
Positive |
Biopsy |
III |
Yes |
Yes |
I |
No |
CR |
57.83 |
|
12 |
F |
2.60 |
Tumor |
Trunk |
>5cm |
no |
Negative |
N/A |
Complete
resection |
II |
No |
No |
- |
No |
CR |
197.41 |
|
13 |
M |
8.26 |
Tumor |
H&N |
<5cm |
no |
N/A |
N/A |
Partial
resection |
III |
No |
No |
|
No |
CR |
31.54 |
|
14 |
M |
1.45 |
Tumor |
Limb |
>5cm |
no |
N/A |
Positive |
Biopsy |
III |
Yes |
No |
|
No |
CR |
110.72 |
|
15 |
F |
36.41 |
Tumor |
Limb |
<5cm |
no |
N/A |
Negative |
Complete
resection |
I |
No |
No |
- |
No |
CR |
1.12 |
H&N: Head and Neck; PD: Progressive
Disease; CR: Complete Remission; Mtts: Metastasis; SD: Stable Disease; OS:
Overall Survival; N/A: Not Available Sx: Surgery.
*Recurrent
fusion involving RAF1; **metastatic.