title: Desmoid fibromatosis in pediatric patients: management based on a retrospective analysis of 59 patients and a review of the literature.
abstract: Background. 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.
authors.affiliation: Pediatric Oncology Department, Hôpital de la mère et de l'enfant, 8 rue Dominique Larrey, 87042 Limoges, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Oudot
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.forename: Véronique
authors.initials: V
authors.lastname: Minard-Colin
authors.forename: Jean
authors.initials: J
authors.lastname: Michon
authors.forename: Pierre
authors.initials: P
authors.lastname: Mary
authors.forename: Christophe
authors.initials: C
authors.lastname: Glorion
authors.forename: Sylvie
authors.initials: S
authors.lastname: Helfre
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Habrand
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin