Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Abstract EN

Only limited data are available concerning desmoid tumor in children.

Methods.

Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed.

Results.

Median age was 6 years (range, 0–15).

Tumors mainly involved the limbs (42%).

Five cases occurred in a context of genetic disorder.

Surgery was first-line treatment in 80% of cases.

Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases.

Various adjuvant therapies were used.

Overall response to all systemic therapies was 33%.

Thirty-eight patients developed one or more recurrences or progressions.

After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR.

Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context.

Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively.

In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS.

Conclusions.

When surgery is required, surgical margins must be negative.

Low-dose chemotherapy can be proposed as adjuvant therapy.

Prospective trials must be developed to evaluate long-term response and side effects.