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

Caroline Oudot,Jean-Louis Habrand,Christophe Glorion,Sylvie Helfre,Daniel Orbach,Odile Oberlin,Jean Michon,Pierre Mary,Véronique Minard-Colin

doi:10.1155/2012/475202

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.

Highlights

Desmoid fibromatosis or desmoid tumor (DT), called deep fibromatosis or aggressive fibromatosis, is a rare disease
The age distribution of patients with a primary in a site other than the head and neck was well balanced: 20 patients were under the age of 10 and 20 were older than 10
This retrospective study with a long followup of a large cohort of pediatric patients with DT confirms the treatment difficulties encountered with this benign tumor, as complete resection is rare, loco-regional progression is frequent and this disease tends to become “chronic,” even in children

Summary

Introduction

Desmoid fibromatosis or desmoid tumor (DT), called deep fibromatosis or aggressive fibromatosis, is a rare disease. The etiology is unknown, but DT may be associated with trauma and occurs as a feature of Gardner’s syndrome or familial adenomatous polyposis coli (FAP) [4, 5]. They have a locally aggressive behavior with no distant metastases, but a tendency for local recurrence after therapy [5]. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. Prospective trials must be developed to evaluate long-term response and side effects

Methods

Results

Conclusion