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Pazopanib is an active treatment in desmoid tumour/aggressive fibromatosis.


ABSTRACT: 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.

SUBMITTER: Martin-Liberal J 

PROVIDER: S-EPMC4176486 | biostudies-literature | 2013 Nov

REPOSITORIES: biostudies-literature

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Pazopanib is an active treatment in desmoid tumour/aggressive fibromatosis.

Martin-Liberal Juan J   Benson Charlotte C   McCarty Heather H   Thway Khin K   Messiou Christina C   Judson Ian I  

Clinical sarcoma research 20131126 1


<h4>Background</h4>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.<h4>Cases presentation</h4>We report the outcome of two patients affected by progressive DT/AF treated with the angiogenesis inhibitor pazopanib in two different institutions. Both patients ach  ...[more]

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