Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature
Table 2
Details on radiation therapy in 21 patients with desmoid tumor.
Disease status at time of RT
Timing of RT
RT dose (Gy)
Long-term status
Site of relapse
Time to relapse or progression after RT (months)
Delayed second RT
Microscopic disease ()
First line
54.4
Relapse
Out
22
—
First line
50
Relapse
In
12
—
First line
45
Relapse
In
10
—
After relapse
45
CRem
—
No
After relapse
55
CRem
—
No
After relapse
50
CRem
—
No
After relapse
50
CRem
—
No
After relapse
45
CRem
—
No
Progressive disease
50
CRem
—
No
Progressive disease
52
Progressive disease
In
20
No
Macroscopic disease ()
First line
50
CRem
—
—
First line
45
CRem
—
—
First line
45
CRem
—
—
First line
50
Relapse
Out
45
—
After relapse
51
CRem
—
No
After relapse
50
CRem
—
No
After relapse
50
CRem
—
Yes
After relapse
50
Relapse
Out
4
No
After relapse
45*
Relapse
In
22
Yes
After relapse
50
Relapse
Out
8
Yes
After relapse
35*
Progressive disease
In
0
Yes
Progressive disease
40*
Progressive disease
In
3
No
Negative ()
After relapse
44
Relapse
In
17
No
Unknown ()
First line
45
CRem
—
—
First line
46
Relapse
Out
0
—
Abbreviations: RT dose: radiation dosage. Microscopic disease: R1 margins after surgery. Macroscopic disease: R2 margins after surgery or no prior surgery (*). Negative disease: surgery with negative margins R0. Unknown disease: status of margins unknown after surgery. Relapse In: relapse in radiation fields. Relapse Out: relapse outside of radiation fields. CRem: complete remission.