Aggressive fibromatosis of the head and neck in the pediatric population

Abstract

Background: Aggressive fibromatosis, previously referred to as desmoid tumor, is a rare tumor. It is classified as benign, however, has a high potential for local invasion and recurrence. It is most commonly found in the abdomen, with an origin in the head and neck accounting for less than a quarter of the total cases.

Methods: Literature review using PubMed and OVID and a combination of the words, "desmoid", "fibromatosis", "pediatric", "children", and "head and neck".

Results: Annual occurrence is 0.2 - 0.4 per 100,000, with origins in the head and neck accounting for 10-25%. Since 1954, 97 cases of pediatric head and neck fibromatosis were reported. The age range was from birth to 16 years, with the average being 4 years and 5 months old. The overwhelming majority of tumors were of the mandible (38%). 74% patients underwent a large resection as the primary treatment modality. The most common treatment complication was tumor recurrence (16%).

Conclusions: Fibromatosis is a rare tumor of muscoloaponeurotic origin. Surgery is the mainstay of treatment; however recurrence rates are high, even despite negative surgical margins.

Keywords: Desmoid tumor; Fibromatosis.