Original ArticleTreatment of Aggressive Fibromatosis: the Experience of a Single Institution
Introduction
Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour. It virtually never metastasises, but can cause local destruction and organ dysfunction through infiltration of blood vessels and/or nerves. Radical surgery is paramount in local control, but because of the tumour's infiltrative nature, positive surgical margins and, hence, tumour recurrence are common findings 1, 2. Almost 100 years ago, James Ewing proposed treating inoperable tumours with radiation [3]. At that time, the precise role of radiotherapy was still not clearly defined [4]. Radiotherapy may be given as adjuvant treatment to decrease local recurrence, especially when the surgical margin is positive, or as a single treatment in cases where surgery will result in severe morbidity or disruption of organ function [5]. Nuyttens et al.[6], in a comparative review between surgery and radiotherapy, found that local control can approach 83% for primary radiotherapy.
The aim of the present study was to analyse our treatment results in this rare tumour type and to identify the important prognostic factors that could affect treatment outcome.
Section snippets
Materials and Methods
This was a retrospective analysis of patients with primary and recurrent aggressive fibromatosis treated at King Faisal Specialist Hospital (KFSH) between 1990 and 2006.
This study was approved by the research accreditation and ethical committee at KFSH in 2007.
Overall Outcome
For the whole group (taking all patients' primary and recurrent cases at presentation), with a median follow-up of 7.3 years, there were 10 (18.5%) local recurrences, all of which were within the original primary site or within the irradiated field. The actuarial local control rate at 5 years was 75% and at 10 years 67% (Fig. 1).
The univariate regression analysis of factors potentially affecting local control is shown in Table 2. In the univariate analysis, patients with completely resected
Discussion
Aggressive fibromatosis is a locally aggressive tumour, with a local recurrence rate that may reach as high as 70–90%, especially after incomplete resection 9, 10.
The treatment of fibromatosis usually consists of adequate surgery with a wide surgical margin (the definition of this margin is still contradictory), plus or minus postoperative radiotherapy 1, 11, 12. Chemotherapy, non-steroidal anti-inflammatory drugs or tamoxifen have been used, with limited published data 13, 14. In case surgery
Conclusion
Overall survival for aggressive fibromatosis is usually high and most deaths may not be related to the disease itself. Local control rates for primary vs recurrent disease were comparable in our data set. Radiotherapy and adequate surgery are very effective in radical treatment for aggressive fibromatosis, but still more data are needed to define when and who should be treated with radiotherapy.
Acknowledgment
We would like to thank Dr Abdelmoneim Eldaly from the Biostatistics Department for his help with the statistics for this study.
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