Age-based treatment of aggressive fibromatosis in the head and neck region.

PURPOSE: To review our experience regarding the difference in management and treatment outcomes of aggressive fibromatosis of the head and neck region in children and adults, emphasizing, in particular, the role of conservative surgery in comprehensive treatment strategies. PATIENTS AND METHODS: A retrospective analysis of patients with aggressive fibromatosis was performed during a 5-year period (2008 to 2012). Nine patients were enrolled in the present study, including 5 children (age, <18 years) and 4 adults (age, >18 years). All patients underwent surgical intervention and were treated by surgical resection with different surgical margins. Adjuvant low-dose chemotherapy and radiotherapy were given to pediatric and adult patients, respectively, with macroscopically or microscopically positive surgical margins. RESULTS: All 5 pediatric patients (3 females and 2 males) received low-dose chemotherapy after conservative surgical resection (in 4 patients, microscopically incomplete resection; and in 1 patient, macroscopic residual tumor). Of the 4 adults (3 females and 1 male), 2 underwent complete surgical resection and 2 underwent surgery and postoperative radiotherapy (1 patient had microscopically suspected residual tumor and 1 had macroscopic residual tumor). The patients were followed up for a period of 7 to 51 months. Two pediatric patients and one adult patient had disease progression after resection and became stable after continued adjuvant therapy. None of the patients had functional or cosmetic defects. All patients had good long-term outcomes, with no disease progression. CONCLUSIONS: For the treatment of aggressive fibromatosis, conservative resection with preservation of form and function should be given greater priority in all age groups. Also, postoperative adjuvant therapy is vital for patients with gross or microscopic residual tumor to obtain progression-free survival.