An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)

Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European D...

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Detalhes bibliográficos
Autores: Kasper, Bernd, Baumgarten, C., García, J., Bonvalot, Sylvie, Haas, R. L., Haller, F., Hohenberger, Peter, Penel, Nicolas, Messiou, C., van der Graaf, W. T., Gronchi, Alesandro, Bauer, Sebastian, Blay, Jean-Yves, van Coevorden, F., Dileo, P., Dürr, H. R., Fiore, M. R., Grünwald, V., Jones, Robin L., Judson, I., Kettelhack, C., Kopeckova, K., Lazar, A. J., Lindner, Lars H., Martín-Broto, Javier, Rutkowski, Piotr, Stacchiotti, Silvia, Stoeckle, E., Valverde, Claudia M., Verhoef, K., Wardelmann, Eva, Wartenberg, M.
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2017
País:España
Recursos:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/182594
Acesso em linha:http://hdl.handle.net/10261/182594
Access Level:acceso abierto
Palavra-chave:Desmoid
Aggressive fibromatosis
EORTC/STBSG
Patient advocacy groups
SPAEN
Treatment algorithm
Descrição
Resumo:Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals’ AND patients’ expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet. In this paper, we focus on new findings regarding the prognostic value of mutational analysis in desmoid-type fibromatosis patients and new systemic treatment options.

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