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