Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature.

Background: Aggressive fibromatosis (AF) is a soft tissue tumor and is rare in childhood, with high potential for local invasiveness and recurrence. General recommendations for the clinical management of pediatric patients with AF remain undetermined.

Methods: The authors retrospectively analyzed 13 children with AF who were diagnosed from 1987 until 2004 in the Erasmus MC-Sophia Children's Hospital, and a review of the pediatric literature was conducted.

Results: Two patients received preoperative chemotherapy with combined vincristine, actinomycin-D, and cyclophosphamide (VAC). All 13 patients underwent surgery. Three of six patients who underwent incomplete resection received adjuvant treatment, two patients received radiotherapy, and one patient received chemotherapy (VAC). The median follow-up was 3.9 years (range, 0.6-14.0 years). Three patients developed recurrent AF, including two recurrences after patients underwent incomplete resection without adjuvant treatment. Secondary resection was performed, which was incomplete in one patient who subsequently received chemotherapy (VAC). At the time of the current report, all 13 patients were in complete remission. Ten pediatric AF studies, including the current study, with a total of 187 patients were reviewed. Incomplete resection was the most important determinant for disease recurrence; in the authors' opinion, the role of adjuvant therapy needs to be studied further.

Conclusions: Primary surgery with negative surgical margins was found to be the most successful primary treatment modality for children with AF. Positive margins after surgery indicated a high risk for disease recurrence. Multicenter, prospective (randomized) trials will be necessary to clarify the role of adjuvant treatment for patients with pediatric AF.