TY - JOUR
T1 - Pleuropulmonary blastoma in children and adolescents
T2 - The EXPeRT/PARTNER diagnostic and therapeutic recommendations
AU - Bisogno, Gianni
AU - Sarnacki, Sabine
AU - Stachowicz-Stencel, Teresa
AU - Minard Colin, V.
AU - Ferrari, Andrea
AU - Godzinski, Jan
AU - Gauthier Villars, Marion
AU - Bien, Ewa
AU - Hameury, Frederic
AU - Helfre, Sylvie
AU - Schneider, Dominik T.
AU - Reguerre, Yves
AU - Lopez Almaraz, Ricardo
AU - Janic, Dragana
AU - Cesen, Maja
AU - Kolenova, Alexandra
AU - Rascon, Jelena
AU - Martinova, Kata
AU - Cosnarovici, Rodica
AU - Pourtsidis, Apostolos
AU - Ben Ami, Tal
AU - Roganovic, Jelena
AU - Koscielniak, Ewa
AU - Schultz, Kris Ann P.
AU - Brecht, Ines B.
AU - Orbach, Daniel
N1 - Publisher Copyright:
© 2021 Wiley Periodicals LLC.
PY - 2021/6/1
Y1 - 2021/6/1
N2 - 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).
AB - 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).
KW - PARTNER
KW - children
KW - pleuropulmonary blastoma
KW - therapeutic recommendations
KW - very rare tumors
UR - http://www.scopus.com/inward/record.url?scp=85103616886&partnerID=8YFLogxK
U2 - 10.1002/pbc.29045
DO - 10.1002/pbc.29045
M3 - Article
C2 - 33826235
AN - SCOPUS:85103616886
SN - 1545-5009
VL - 68
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
IS - S4
M1 - e29045
ER -