Abstract
Background:The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients.Methods:Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids.Results:Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy.Conclusion:For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin.British Journal of Cancer advance online publication, 9 November 2010; doi:10.1038/sj.bjc.6605997 www.bjcancer.com.
Original language | English |
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Pages (from-to) | 37-42 |
Number of pages | 6 |
Journal | British Journal of Cancer |
Volume | 104 |
Issue number | 1 |
DOIs | |
Publication status | Published - 4 Jan 2011 |
Keywords
- desmoid tumour
- desmoid-type fibromatosis
- familial adenomatous polyposis
- management
- AGGRESSIVE FIBROMATOSIS
- RADIATION-THERAPY
- SURGERY
- DIAGNOSIS
- IMATINIB
- OUTCOMES