Aggressive fibromatosis: evidence for a stable phase

Area of Science:

• Oncology
• Surgical Pathology
• Clinical Medicine

Background:

• Aggressive fibromatosis (AF) is a rare, locally infiltrating benign soft tissue tumor.
• Current treatments include surgery, radiotherapy, hormone therapy, and chemotherapy, all associated with morbidity.
• The natural history of AF may involve periods of stable disease, potentially obviating immediate treatment.

Purpose of the Study:

• To investigate the natural history of aggressive fibromatosis.
• To determine the occurrence and duration of stable disease periods in AF patients.
• To evaluate the necessity of immediate treatment in AF management.

Main Methods:

• Retrospective review of 42 patients with aggressive fibromatosis treated at a single institution.
• Analysis of case notes to identify periods of stable disease (defined as no objective progression for ≥6 months).
• Correlation of stable disease episodes with treatment modalities and outcomes.

Main Results:

• All 17 assessable patients (100%) experienced at least one period of stable disease.
• Eight patients had stable disease while undergoing hormonal or cytotoxic therapy.
• Of 23 patients not assessed for stable disease due to surgery, only 2 had persistent disease, both with positive resection margins.

Conclusions:

• Aggressive fibromatosis exhibits a variable natural history with frequent periods of stable disease.
• A less aggressive management strategy for AF may be appropriate.
• Identifying patients likely to experience stable disease could refine treatment decisions.