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

M.H. Nieuwenhuis, E.M. Mathus Vliegen, C.G.M.I. Baeten, F.M. Nagengast, J. van der Bijl, A.D. van Dalsen, J.H. Kleibeuker, E. den 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. Vasen

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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 languageEnglish
Pages (from-to)37-42
Number of pages6
JournalBritish Journal of Cancer
Volume104
Issue number1
DOIs
Publication statusPublished - 4 Jan 2011

Keywords

  • desmoid tumour
  • desmoid-type fibromatosis
  • familial adenomatous polyposis
  • management
  • AGGRESSIVE FIBROMATOSIS
  • RADIATION-THERAPY
  • SURGERY
  • DIAGNOSIS
  • IMATINIB
  • OUTCOMES

Cite this

Nieuwenhuis, M. H., Mathus Vliegen, E. M., Baeten, C. G. M. I., Nagengast, F. M., van der Bijl, J., van Dalsen, A. D., ... Vasen, H. F. (2011). Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients. British Journal of Cancer, 104(1), 37-42. https://doi.org/10.1038/sj.bjc.6605997
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. / Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients. In: British Journal of Cancer. 2011 ; Vol. 104, No. 1. pp. 37-42.
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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.",
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author = "M.H. Nieuwenhuis and {Mathus Vliegen}, E.M. and C.G.M.I. Baeten and F.M. Nagengast and {van der Bijl}, J. and {van Dalsen}, A.D. and J.H. Kleibeuker and {den Dekker}, E. and A.M. Langers and J. Vecht and F.T. Peters and {Van Dam}, R. and {van Gemert}, W.G. and W.N. Stuifbergen and W.R. Schouten and H. Gelderblom and H.F. Vasen",
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Nieuwenhuis, MH, Mathus Vliegen, EM, Baeten, CGMI, Nagengast, FM, van der Bijl, J, van Dalsen, AD, Kleibeuker, JH, den Dekker, E, Langers, AM, Vecht, J, Peters, FT, Van Dam, R, van Gemert, WG, Stuifbergen, WN, Schouten, WR, Gelderblom, H & Vasen, HF 2011, 'Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients.', British Journal of Cancer, vol. 104, no. 1, pp. 37-42. https://doi.org/10.1038/sj.bjc.6605997

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

In: British Journal of Cancer, Vol. 104, No. 1, 04.01.2011, p. 37-42.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients.

AU - Nieuwenhuis, M.H.

AU - Mathus Vliegen, E.M.

AU - Baeten, C.G.M.I.

AU - Nagengast, F.M.

AU - van der Bijl, J.

AU - van Dalsen, A.D.

AU - Kleibeuker, J.H.

AU - den Dekker, E.

AU - Langers, A.M.

AU - Vecht, J.

AU - Peters, F.T.

AU - Van Dam, R.

AU - van Gemert, W.G.

AU - Stuifbergen, W.N.

AU - Schouten, W.R.

AU - Gelderblom, H.

AU - Vasen, H.F.

PY - 2011/1/4

Y1 - 2011/1/4

N2 - 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.

AB - 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.

KW - desmoid tumour

KW - desmoid-type fibromatosis

KW - familial adenomatous polyposis

KW - management

KW - AGGRESSIVE FIBROMATOSIS

KW - RADIATION-THERAPY

KW - SURGERY

KW - DIAGNOSIS

KW - IMATINIB

KW - OUTCOMES

U2 - 10.1038/sj.bjc.6605997

DO - 10.1038/sj.bjc.6605997

M3 - Article

VL - 104

SP - 37

EP - 42

JO - British Journal of Cancer

JF - British Journal of Cancer

SN - 0007-0920

IS - 1

ER -