Extra-abdominal Desmoid Fibromatosis
Clinical
- Over age 5 years by definition
- Nearly always over 12 years
- Most age 15-40
- Not associated with pregnancy
- Not associated with familial polyposis/Gardner syndrome
- Excludes lesions of abdominal wall and abdominal cavity, by definition
- Most common on trunk and proximal extremities
- Rare in feet or hands
- May be multicentric
- Not considered to be metastases
- Frequent recurrence
- 20-75%
- Recurrences may be locally aggressive and destructive
- No metastases
- Deep fibromatoses differ primarily in their clinical association
