Agressive fibromatosis involving the mandible--case report and review of the literature.

Seper L, Bürger H, Vormoor J, Joos U, Kleinheinz J

Abstract

BACKGROUND: Aggressive fibromatosis (AF) involving the mandible is rare, and surgery is often complicated by a high recurrence rate. CASE REPORT: A 4-year-old boy was referred because of a fast growing painless mass which involved the entire left angle of the mandible. Excisional biopsy revealed AF, and local excision of the tumor was performed. Six months after surgery a recurrence was detected. The tumor was determined to be unresectable and the boy was treated with low-dose chemotherapy including methotrexate and vinblastine for 1 year. With combined chemotherapy and surgical debulking, mutilating surgical resection will be delayed as long as possible or until completion of facial growth. CONCLUSION: According to the literature, surgery is the most common treatment of AF in the head and neck region. However, particularly in children, alternative modes of therapy must be considered because of the high recurrence rate and to avoid mutilating operations.