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Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations
Author(s) -
Bisogno Gianni,
Sarnacki Sabine,
StachowiczStencel Teresa,
Minard Colin V.,
Ferrari Andrea,
Godzinski Jan,
Gauthier Villars Marion,
Bien Ewa,
Hameury Frederic,
Helfre Sylvie,
Schneider Dominik T.,
Reguerre Yves,
Lopez Almaraz Ricardo,
Janic Dragana,
Cesen Maja,
Kolenova Alexandra,
Rascon Jelena,
Martinova Kata,
Cosnarovici Rodica,
Pourtsidis Apostolos,
Ben Ami Tal,
Roganovic Jelena,
Koscielniak Ewa,
Schultz Kris Ann P.,
Brecht Ines B.,
Orbach Daniel
Publication year - 2021
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.29045
Subject(s) - medicine , adjuvant radiotherapy , pediatrics , radiation therapy , pulmonary blastoma , adjuvant chemotherapy , population , european union , cancer , surgery , breast cancer , environmental health , lung , business , economic policy
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).