There is no gender or racial preponderance. The abdominal desmoid tumors are often associated with familial polyposis coli or Gardner's syndrome. These tumors are characteristically slow growing. Most relentlessly enlarge. In children, many aggressive fibromatoses will eventually compromise local structures.
Neck lesions are particularly troublesome.
Extremity and abdominal lesions can be massive prior to impinging upon surrounding structures. The mainstay of management is wide local excision whenever possible. However, unacceptably high local recurrence rates have been reported. It is the children in groups 2 and 3 who clearly require adjuvant therapy. It is unclear whether a child with a gross total resection but positive margins should receive further therapy.