Research output

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

Research output: Contribution to journalArticleAcademicpeer-review

Associated researcher

  • Nieuwenhuis, M. H.
  • Mathus Vliegen, E. M.
  • Baeten, C.G.M.I.

  • Nagengast, F. M.
  • van der Bijl, J.
  • van Dalsen, A. D.
  • Kleibeuker, J. H.
  • den 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. F.

Associated organisations


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

    Research areas

  • desmoid tumour, desmoid-type fibromatosis, familial adenomatous polyposis, management, AGGRESSIVE FIBROMATOSIS, RADIATION-THERAPY, SURGERY, DIAGNOSIS, IMATINIB, OUTCOMES
View graph of relations


Original languageEnglish
Pages (from-to)37-42
Number of pages6
JournalBritish Journal of Cancer
Issue number1
Publication statusPublished - 4 Jan 2011