| Aggressive fibromatosis | |
|---|---|
| Classification and external resources | |
MR scan of pelvis - desmoid tumor |
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| DiseasesDB | 29794 |
| eMedicine | article/1060887 |
| MeSH | D018222 |
Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing tumors without any metastatic potential. However, Aggressive Fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ dysfunction. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.[1]
Histologically they resemble low-grade fibrosarcomas,[2] but they are very locally aggressive and tend to recur even after complete resection. There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery.[3]
Risk factors for desmoid disease amongst FAP patients include female gender, a 3' APC mutation, a positive family history and a history of previous abdominal surgery. [4]
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Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal (the last is more common in patients with FAP). It is thought that the lesions may develop in relation to estrogen levels or trauma/operations.
A 3' APC mutation is the most significant risk factor for intra-abdominal desmoid development amongst FAP patients.[5]FAP patients presenting with an abdominal wall desmoid pre-operatively are at an increased risk of developing an intra-abdominal desmoid post-operatively.[6]
Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens and NSAIDs, or chemotherapy.
Current experimental studies are being done with Gleevec (Imatinib) for treatment of Desmoid tumors, and show promising success rates.
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