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

  • Caroline Oudot,1 Daniel Orbach,2 Véronique Minard-Colin,3 Jean Michon,2 Pierre Mary,4 Christophe Glorion,5 Sylvie Helfre,6 Jean-Louis Habrand,7 and Odile Oberlin3

  • Erratum Erratum to ‘‘Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature’’

  • 1 Pediatric Oncology Department, Hopital de la Mere et de l’Enfant, 87042 Limoges, France 2 Adolescent and Pediatric Oncology Department, Institut Curie, 75005 Paris, France 3 Adolescent and Pediatric Oncology Department, Institut Gustave Roussy, 94800 Villejuif, France 4 Pediatric Surgery Department, Hopital Trousseau, 75012 Paris, France 5 Pediatric Surgery Department, Hopital Necker-Enfants-Malades, 75012 Paris, France 6 Radiotherapy Department, Institut Curie, 75005 Paris, France 7 Radiotherapy Department, Centre Hospitalier Universitaire de Caen, 14000 Caen, France Correspondence should be addressed to Caroline Oudot; oudot caroline@yahoo.fr

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Introduction

Caroline Oudot,1 Daniel Orbach,2 Véronique Minard-Colin,3 Jean Michon,2 Pierre Mary,4 Christophe Glorion,5 Sylvie Helfre,6 Jean-Louis Habrand,7 and Odile Oberlin3. Erratum Erratum to ‘‘Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature’’

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