Table 1. Characteristics of patients with extra-abdominal fibromatosis by β-catenin genotype.
| CTNNB1 | Total | WT | Mutated | 41A | 45F | 45P | del |
|---|---|---|---|---|---|---|---|
| characteristics | n | n | n | n | n | n | |
| N | 101 | 13 | 88 | 40 | 37 | 9 | 2 |
| Age | |||||||
| Median, years | 37 | 39.5 | 37.5 | 36.7 | 37 | 45.5 | 43 |
| Range | 0.1–77 | 0.5–66 | 0.1–77 | 10–73 | 0.1–77 | 14–65 | 19–68 |
| Sex | |||||||
| Male | 36 | 4 | 32 | 14 | 15 | 3 | — |
| Female | 65 | 9 | 56 | 26 | 22 | 6 | 2 |
| Presentation | |||||||
| Primary | 57 | 12 | 45 | 16 | 20 | 8 | 1 |
| Relapse | 40 | 1 | 39 | 22 | 15 | 1 | 1 |
| NA | 4 | — | 4 | 2 | 2 | — | — |
| Tumour site | |||||||
| Head/Neck | 8 | 1 | 7 | 2 | 5 | — | — |
| Trunk | 54 | 5 | 49 | 20 | 19 | 8 | 2 |
| Limb | 37 | 7 | 30 | 17 | 12 | 1 | — |
| NA | 2 | — | 2 | 1 | 1 | — | — |
| Tumour size, mm | |||||||
| Median, mm | 80 | 40 | 80 | 80 | 85 | 75 | 40 |
| Range | 10–300 | 15–120 | 10–300 | 21–300 | 25–200 | 25–190 | 10–70 |
| Therapy | |||||||
| Surgery | 101 | 13 | 88 | 40 | 37 | 9 | 2 |
| Radiation therapy | 18 | 1 | 17 | 7 | 8 | 1 | 1 |
| Medical therapy | 8 | 1 | 7 | 3 | 4 | — | — |
| Surgical margin | |||||||
| RO | 42 | 9 | 33 | 14 | 13 | 5 | 1 |
| R1 | 33 | 3 | 30 | 15 | 12 | 3 | — |
| R2 | 8 | — | 8 | 1 | 5 | 1 | 1 |
| NA | 18 | 1 | 17 | 10 | 7 | — | — |
| Outcome | |||||||
| Relapse | 51 | 1 | 50 | 22 | 23 | 4 | 1 |
Patient clinical data and β-catenin genotype tumour data from the series of extra-abdominal fibromatosis tumours. WT, wild β-catenin gene (exon 3) del, deletion in exon 3; 41A, 45F, 45P, mutated residues; n, number of patients; NA, not available; mm, millimiter; R0, R1, and R2, quality of surgical margins according to the UICC-R classification system.