Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Area of Science:

• Pediatric Oncology
• Rare Cancers
• Tumor Biology

Background:

• Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm predominantly affecting young children.
• Clinical presentation may include advanced upper-abdominal tumors and elevated serum alpha-fetoprotein levels, necessitating histopathological confirmation.
• Complete surgical resection is the primary treatment modality for PBL.

Purpose of the Study:

• To establish international consensus recommendations for the diagnosis and treatment of pediatric pancreatoblastoma.
• To provide guidance for managing unresectable or metastatic cases with neoadjuvant chemotherapy followed by delayed surgery.
• To consolidate expertise from the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the PARTNER project.

Main Methods:

• Development of international consensus recommendations.
• Review of diagnostic criteria and treatment strategies for pediatric pancreatoblastoma.
• Inclusion of data from the EU-funded PARTNER project registry.

Main Results:

• Established comprehensive guidelines for PBL diagnosis, including histopathological confirmation.
• Outlined treatment algorithms, emphasizing complete surgical resection as the mainstay.
• Recommended neoadjuvant chemotherapy for unresectable or metastatic PBL to facilitate delayed surgical resection.

Conclusions:

• International consensus recommendations for pediatric pancreatoblastoma diagnosis and treatment have been established.
• These guidelines aim to improve outcomes for children with this rare malignancy.
• Multidisciplinary collaboration and adherence to standardized protocols are crucial for optimal patient management.