Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations
Gianni Bisogno, Sabine Sarnacki, Teresa Stachowicz‐Stencel, Veronique Minard Colin, Andrea Ferrari, Jan Godziński, Marion Gauthier Villars, Ewa Bień, Frédéric Hameury, Sylvie Helfré, Dominik T. Schneider, Yves Réguerre, Ricardo López Almaraz, Dragana Janić, Maja Česen, Alexandra Kolenová, Jelena Rascon, Ката Мартинова, Rodica Cosnarovici, Apostolos Pourtsidis, Tal Ben Ami, Jelena Roganović, Ewa Kościelniak, Kris Ann P. Schultz, Ines B. Brecht, Daniel Orbach
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).