Clinical management of pediatric aggressive fibromatosis involving the mandible.

Background: Pediatric aggressive fibromatosis (AF) is a rare, benign tumor with locally infiltrative growth. Therefore, how to prevent reoccurrence while maintaining the mandible contour and continuity as much as possible is very important when the mandible is involved.

Procedure: We selected 10 pediatric patients with AF involving the mandible in our department between January 2001 and December 2011. Patient clinical data, including patient characteristics, symptoms at presentation, management, and treatment outcome, were reviewed.

Results: Patients' ages ranged from 3 to 16 years with six males and four females. According to imaging, there were three cases where the tumor adhered to the mandible; the periosteum was resected with the tumor, followed by cryotherapy or cauterization. Another five cases involved the destruction of the periosteum and the cortical plate. Resection of the involved mandible with the tumor was performed, and the margin was trimmed. In the last two cases, the tumor had invaded the whole ramus, and the immediate iliac graft was operated on after resecting the lesions. Neither radiotherapy (RT) nor chemotherapy was used. There was no recurrence or contour defect of the face. The function of the mandible was not affected, and only one case showed a slight limitation when opening the mouth.

Conclusions: For the treatment of pediatric AF, we recommend complete tumor resection. As for the involved mandible, preserving the mandible contour and continuity as much as possible and providing adjunctive therapy, such as cryotherapy or cauterization, are vital. RT is not recommended.