Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

• DOI: 10.1002/pbc.29112
• Publication Date: 2021-06-26T17:18:16Z
• Authors: Ewa Bien; Jelena Roganovic; Malgorzata A. Kra...; Jan Godzinski; Daniel Orbach; Giovanni Cecchetto; Winfred Barthlen; Anne‐Sophie Defac...; Andrea Ferrari; Christopher B. We...; Ines B. Brecht; Dominik T. Schneider; Gianni Bisogno; Alexandra Kolenova; Tal Ben‐Ami; Kata Martinova; Calogero Virgone; Teresa Stachowicz...; Denis Kachanov; Yves Reguerre
• Keywords: Surgical resection; Paediatric surgery; Consensus conference

Abstract Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper‐abdominal tumor accompanied by elevated serum α‐fetoprotein levels in child suggest PBL, however histopathological confirmation mandatory. The mainstay of the treatment complete surgical resection. Unresectable and/or metastatic PBL may become amenable delayed surgery after neoadjuvant chemotherapy. This manuscript presents international consensus recommendations for diagnosis and children with established European Cooperative Study Group Pediatric Rare Tumors (EXPeRT) within EU‐funded PARTNER (Paediatric Network – Registry) project.