Deep fibromatosis
Deep fibromatosis
Updated: 08/18/2021
© Jun Wang, MD, PhD
General features
- AKA desmoid tumor
- Clonal proliferation of fibroblasts/myofibroblasts with an infiltrating pattern
- May be associated with familial adenomatous polyposis (Gardner syndrome)
- More common in young women
- More common in man if associated with familial adenomatous polyposis (Gardner syndrome)
- Locally aggressive
- Prognosis associated with age, size and site
- May be fatal due to local effects, esp. in head and neck
Clinical presentations
- Usually painless mass
- Dysfunction of surrounding tissue/organ due to infiltrative growth
- Three types
Abdominal fibromatosis:
Arises within abdominal wall during or after
pregnancy
May be associated with cesarean section scar
Extraabdominal fibromatosis:
Arises outside abdomen and abdominal wall,
usually in muscles of shoulder, chest wall, back and thigh, equal gender
frequencies
Intraabdominal fibromatosis:
Mesenteric, pelvic or retroperitoneal locations,
associated with Gardner's syndrome
(familial adenomatous polyposis, multiple osteomas)
Often post-surgical
Key morphological features
- Bipolar fibroblasts and myofibroblasts in collagenous stroma
- NO atypia
Genetic abnormalities
- Beta-catenin or APC mutation
- May have trisomy 20 or 8
Treatment
- Wide excision
Back to contents
Comments
Post a Comment