Background: Desmoid 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 Presentation: We 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.

Conclusions: Pazopanib is an active treatment in DT/AF. It is the first time this has been reported.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4176486PMC
http://dx.doi.org/10.1186/2045-3329-3-13DOI Listing

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