Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Gianni Bisogno, Sabine Sarnacki, Teresa Stachowicz-Stencel, V. Minard Colin, Andrea Ferrari, Jan Godzinski, Marion Gauthier Villars, Ewa Bien, Frederic Hameury, Sylvie Helfre, Dominik T. Schneider, Yves Reguerre, Ricardo Lopez Almaraz, Dragana Janic, Maja Cesen, Alexandra Kolenova, Jelena Rascon, Kata Martinova, Rodica Cosnarovici, Apostolos PourtsidisTal Ben Ami, Jelena Roganovic, Ewa Koscielniak, Kris Ann P. Schultz, Ines B. Brecht, Daniel Orbach

    Résultats de recherche: Contribution à un journalArticleRevue par des pairs

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    Résumé

    Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).

    langue originaleAnglais
    Numéro d'articlee29045
    journalPediatric Blood and Cancer
    Volume68
    Numéro de publicationS4
    Les DOIs
    étatPublié - 1 juin 2021

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