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Evaluation of Management of Desmoid Tumours Associated with Familial Adenomatous Polyposis in Dutch Patients
source: British journal of cancer
authors: Nieuwenhuis M H, Mathus-Vliegen E M, Baeten C G, Nagengast F M, van der Bijl J, van Dalsen A D, Kleibeuker J H, Dekker E, Langers A M, Vecht J, Peters F T, van Dam R, van Gemert W G, Stuifbergen W N, Schouten W R, Gelderblom H, Vasen H Fsummary/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.organization: The Netherlands Foundation for the Detection of Hereditary Tumours
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