Clinical Study
Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature
Table 1
Clinical characteristics, treatment modalities, and outcome of the 59 patients with desmoid tumors.
| | Quality of surgical margins | Total (number of patients) | IRS I | IRS II | IRS III |
Unknown IRS group | | R0 | R1 | R2 resection | Biopsy | | | | | | | | |
| | Patients and tumor | | | | | | | | Gender | | | | | | | | Male | 36 | 2 | 10 | 15 | 7 | 2 | | Female | 23 | 1 | 9 | 8 | 5 | 0 | | Age at diagnosis (years) | | | | | | | | Median | 6.75 | 5.9 | 12.8 | 6.08 | 9.03 | 6 | | Range | 0–15 | 2–14 | 1–14 | 0–15 | 0–15 | 5–7 | | Primary | | | | | | | | Head and neck | 17 | — | 4 | 8 | 4 | 1 | | Wall trunk | 9 | 1 | 3 | 4 | 1 | — | | Deep trunk/visceral | 6 | 1 | 1 | 2 | 2 | — | | Limbs | 25 | 1 | 10 | 9 | 4 | 1 | | Multiple sites | 2 | — | 1 | — | 1 | — | | Predisposing conditions | | | | | | | | Gardner’s/FAP | 5 | | | | | | | Local surgical trauma | 3 | | | | | | | First-line treatment | | | | | | | | (i) Initial surgery | 47 | 3 | 19 | 23 | — | 2 | | No further therapy | 35 | 3 | 16 | 16 | — | — | | Adjuvant therapy: | | | | | | | | RT | 9 | — | 3 | 4 | — | 2 | | CT | 3 | — | — | 3 | — | — | | (ii) No initial surgery | 12 | — | — | — | 12 | — | | Neoadjuvant therapy | | | | | | | | Medical treatment then surgery | 7 | — | — | — | 7 | — | | Medical treatment alone | 1 | — | — | — | 1 | — | | Wait-and-see strategy | 4 | — | — | — | 4 | — | | Outcome | | | | | | | | Relapse/Progression | 38 pts | 2 pts | 16 pts | 15 pts | 4 pts | 1 pt | | 5-year PFS | 39% | 33% | 16% | 48% | — | | Death | 7 pts | 0 | 3 pts | 3 pts | 1 pt | 0 |
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Abbreviations: RT: radiation therapy; CT: conventional chemotherapy; PFS: progression-free survival; : number of patients.
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