| Literature DB >> 33826235 |
Gianni Bisogno1, Sabine Sarnacki2, Teresa Stachowicz-Stencel3, V Minard Colin4, Andrea Ferrari5, Jan Godzinski6, Marion Gauthier Villars7, Ewa Bien3, Frederic Hameury8, Sylvie Helfre9, Dominik T Schneider10, Yves Reguerre11, Ricardo Lopez Almaraz12, Dragana Janic13, Maja Cesen14, Alexandra Kolenova15, Jelena Rascon16, Kata Martinova17, Rodica Cosnarovici18, Apostolos Pourtsidis19, Tal Ben Ami20, Jelena Roganovic21, Ewa Koscielniak22, Kris Ann P Schultz23, Ines B Brecht24, Daniel Orbach25.
Abstract
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).Entities:
Keywords: PARTNER; children; pleuropulmonary blastoma; therapeutic recommendations; very rare tumors
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Year: 2021 PMID： 33826235 DOI： 10.1002/pbc.29045
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN： 1545-5009 Impact factor: 3.838