Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

M H Nieuwenhuis, E M Mathus-Vliegen, C G Baeten, F M Nagengast, J van der Bijl, A D van Dalsen, J H Kleibeuker, E Dekker, A M Langers, J Vecht, F T Peters, R van Dam, W G van Gemert, W N Stuifbergen, W R Schouten, H Gelderblom, H F A Vasen, M H Nieuwenhuis, E M Mathus-Vliegen, C G Baeten, F M Nagengast, J van der Bijl, A D van Dalsen, J H Kleibeuker, E Dekker, A M Langers, J Vecht, F T Peters, R van Dam, W G van Gemert, W N Stuifbergen, W R Schouten, H Gelderblom, H F A Vasen

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.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Progression-free interval after primary surgical (black line) and non-surgical (grey line) treatment for mesenterial desmoid tumours in FAP patients (log-rank test, P=0.163).
Figure 2
Figure 2
Progression-free interval after primary treatment for extra-abdominal and abdominal wall desmoid tumours in FAP patients.
Figure 3
Figure 3
Progression-free interval after NSAIDs (n=12, black line), and hormonal therapy or combination therapy (n=11, grey line), irrespective of previous surgery (log-rank test, P=0.111).

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