Management outcome of benign and aggressive fibromatoses of the head and neck

INTRODUCTION: Fibromatosis are uncommon, non-metastasizing fibrous tumors of unknown pathogenesis. A number of characteristics including their local invasiveness. recurrence after surgical excision, and occurrence at multiple sites makes these tumors difficult to treat effectively. Superficial fibromatosis tend to run a benign course while deep fibromatosis (desmoids) have a more rapid growth and aggressive behavior. METHODS: A retrospective analysis of the pathology, treatment, and outcome of patients having either benign or aggressive fibromatosis was conducted. A search of our institutional database identified 36 patients with benign (fibroma or fibromyxoma) and 54 patients with aggressive (desmoids or fibrosarcoma grade 1) fibromatoses. RESULTS: Of the 36 patients with benign lesions ( 14 males, 22 females; median age 49 years, range 1-79 years), 30 had surgical resection, 5 were not treated and 1 was given chemotherapy. Thirty-four patients remain alive with a mean follow-up of 3.7 years. The 2 deaths were not related to tumor recurrence. The 54 patients with aggressive tumors (30 males, 24 females; median age 34 years, range 1-88 years) underwent many different treatments including surgery (23 patients), radiotherapy (3), chemotherapy (3) and combination of surgery and radiotherapy (14), surgery and chemotherapy (7), and surgery, radiotherapy and chemotherapy (1). Three patients were not treated. Forty-three patients are alive after a mean follow-up of 8.3 years. Five of the eleven patients (45%) who died were not treated or treated with only a single modality. CONCLUSIONS: Benign fibromatoses are effectively managed with surgery alone. Multimodality therapy has improved the outcome of patients with aggressive tumors.