Fig. 1The cut section of the abdominal tumor reveals a grayish-white solid mass with partially irregular edges (arrows).
Fig. 2Histologic findings. (A, B) The tumor reveals long fascicles of bland spindle-shaped fibroblasts with collagen deposition. (C) The tumor shows infiltrative growth and the surgical margins are positive. (D, E) The tumor cells show nuclear and cytoplasmic β-catenin immunostaining.
Table 1.Published series of childhood fibromatosis cases
Reference |
No. of patients |
Site of involvement
|
Detailed site in trunk |
H/N |
Limb |
Trunk |
Ayala et al. [4] |
25 |
8 |
13 |
4 |
Abdominal wall (1), NA (3) |
Scougall et al. [13] |
8 |
0 |
4 |
4 |
Pelvis (4) |
Rao et al. [10] |
20 |
7 |
7 |
6 |
Back (1), NA (5) |
Atahan et al. [3] |
4 |
0 |
1 |
3 |
Shoulder (1), gluteal region (2) |
Faulkner et al. [6] |
63a
|
11 |
39 |
8 |
Abdominal wall (1), intra-abdominal type (3), NA (4) |
Skapek et al. [15] |
10 |
1 |
6 |
3 |
Chest wall (3) |
Refsum et al. [11] |
2 |
0 |
0 |
2 |
Lower abdominal wall (2) |
Reich et al. [12] |
5 |
0 |
4 |
1 |
Chest wall (1) |
Spiegel et al. [16] |
18 |
5 |
12 |
1 |
Buttock (1) |
Merchant et al. [9] |
13 |
4 |
2 |
7 |
Abdominal wall (1), paraspinal or chest wall (6) |
Jain et al. [7] |
1 |
0 |
0 |
1 |
Abdominal wall (1) |
Buitendijk et al. [5] |
13 |
5 |
4 |
4 |
Back (2), NA (2) |
Sharma et al. [14] |
10 |
10 |
0 |
0 |
- |
Ademuyiwa et al. [2] |
1 |
0 |
0 |
1 |
Abdominal wall (1) |
Meazza et al. [8] |
94 |
31 |
38 |
25 |
Intra-abdominal type (7), NA (18) |
Present case |
1 |
0 |
0 |
1 |
Abdominal wall (1) |
Total no. (%) |
288 |
82 |
130 |
71 |
Abdominal wall (8, 2.8%) |
Table 2.Characteristics of childhood abdominal fibromatosis
Reference |
Age/Sex |
Tumor size (cm) |
Tumor margin |
R margin |
Histology |
Treatment |
Follow-up |
Jain et al. [7] |
18 mo/M |
4 × 3 |
Unencapsulated |
NA |
Typical |
OP |
3 yr, NR |
Refsum et al. [11] |
5 yr/M |
6 × 5 |
Unencapsulated |
Positive |
Typical |
OP |
7 yr, NR |
|
4.5 yr/M |
6.5 × 5.5 |
Unencapsulated |
Positive |
Typical |
OP |
15 mo, NR |
Ademuyiwa et al. [2] |
5 yr/F |
6 |
Unencapsulated |
Negative |
NA |
OP |
12 wk, NR |
Merchant et al. [9] |
15 yr/F |
NA |
NA |
NA |
NA |
OP, CTx, RTx |
53 mo, dead |
Faulkner et al. [6] |
18 yr/NA |
NA |
NA |
Positive |
NA |
OP, CTx |
Recurrence |
Present case |
15 mo/M |
4.3 × 4.1 |
Unencapsulated |
Positive |
Typical |
OP, CTx |
7 mo, suspicious of recurrence |