Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations.
Ewa BienJelena RoganovicMałgorzata Anna KrawczykJan GodzinskiDaniel OrbachGiovanni CecchettoWinfred BarthlenAnne-Sophie DefachellesAndrea C FerrariChristopher B WeldonInes B BrechtDominik T SchneiderGianni BisognoAlexandra KolenovaTal Ben AmiKata MartinovaCalogero VirgoneTeresa Stachowicz-StencelDenis KachanovYves ReguerrePublished in: Pediatric blood & cancer (2022)
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 a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international 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.
Keyphrases
- neoadjuvant chemotherapy
- clinical practice
- locally advanced
- young adults
- squamous cell carcinoma
- minimally invasive
- lymph node
- small cell lung cancer
- emergency department
- sentinel lymph node
- mental health
- intensive care unit
- coronary artery bypass
- quality improvement
- rectal cancer
- radiation therapy
- hiv testing
- low grade
- acute coronary syndrome
- coronary artery disease
- hepatitis c virus
- middle aged
- surgical site infection
- hiv infected