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Clinical Sarcoma Research

, 3:13

First Online: 26 November 2013Received: 12 November 2013Accepted: 21 November 2013DOI: 10.1186-2045-3329-3-13

Cite this article as: Martin-Liberal, J., Benson, C., McCarty, H. et al. Clin Sarcoma Res 2013 3: 13. doi:10.1186-2045-3329-3-13


BackgroundDesmoid tumours-aggressive fibromatosis DT-AF are infrequent soft-tissue neoplasms. They usually behave as indolent diseases. However, they may grow locally infiltrating or compressing adjacent structures. The role of local treatment is limited and only a few drugs have shown activity.

Cases presentationWe report the outcome of two patients affected by progressive DT-AF treated with the angiogenesis inhibitor pazopanib in two different institutions. Both patients achieved dramatic improvement in their symptoms and radiological signs of response. The clinical benefit lasted for more than 1 year and it is still ongoing.

ConclusionsPazopanib is an active treatment in DT-AF. It is the first time this has been reported.

KeywordsAggressive fibromatosis Desmoid tumour Imatinib Pazopanib PDGFR Pegylated doxorubicin Sorafenib VEGFR Electronic supplementary materialThe online version of this article doi:10.1186-2045-3329-3-13 contains supplementary material, which is available to authorized users.

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Autor: Juan Martin-Liberal - Charlotte Benson - Heather McCarty - Khin Thway - Christina Messiou - Ian Judson


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