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Sarcoma
Volume 2012 (2012), Article ID 475202, 9 pages
http://dx.doi.org/10.1155/2012/475202
Clinical Study

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

1Pediatric Oncology Department, Hôpital de la mère et de l'enfant, 8 rue Dominique Larrey, 87042 Limoges, France
2Adolescent and Pediatric Oncology Department, Institut Curie, 75005 Paris, France
3Pediatric Department, Institut Gustave Roussy, 94800 Villejuif, France
4Pediatric Surgery Department, Hôpital Trousseau, 75012 Paris, France
5Pediatric Surgery Department, Hôpital Necker-Enfants-Malades, 75012 Paris, France
6Radiotherapy Department, Institut Curie, 75005 Paris, France
7Radiotherapy Department, Centre Hospitalier Universitaire de Caen, 14000 Caen, France

Received 29 February 2012; Revised 14 May 2012; Accepted 14 June 2012

Academic Editor: C. Fisher

Copyright © 2012 Caroline Oudot et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.