Bien 2011: pancreatoblastoma, a report from the European cooperative study group for paediatric rare tumours (EXPeRT)
Pooling children treated across Europe over a decade, this study showed that complete surgical removal is what cures pancreatoblastoma, that cisplatin and doxorubicin chemotherapy can shrink tumours that cannot be removed at first, and that about four in five children survive.
Overview
Retrospective multinational series from the EXPeRT group of children with pancreatoblastoma treated in France, Germany, Italy, Poland and the United Kingdom between 2000 and 2009. Most tumours arose in young children and were large at diagnosis; alpha-fetoprotein was raised in the majority and served as a tumour marker.
Complete resection, either upfront or after neoadjuvant chemotherapy, was the strongest determinant of outcome. Cisplatin and doxorubicin (PLADO) was the most active regimen, achieving responses that allowed delayed resection. Event-free survival was about 60 percent and overall survival close to 80 percent at five years, with relapses salvageable in some children.
- Complete resection was the strongest determinant of cure; neoadjuvant chemotherapy made delayed resection possible.
- Cisplatin plus doxorubicin was the most active regimen.
- Five-year event-free survival about 60 percent and overall survival close to 80 percent.
The EXPeRT recommendations, surgery when feasible and PLADO chemotherapy for unresectable or metastatic disease, remain the treatment framework for childhood pancreatoblastoma.
- Retrospective series of a few dozen children with heterogeneous treatment.
- Adult pancreatoblastoma was not included and behaves worse.
Similar pages
not linked directly; found by shared links- Key paperDhebri 2004: diagnosis, treatment and outcome of pancreatoblastoma
- Key paperKlimstra 1995: pancreatoblastoma, a clinicopathologic study and review of the literature
- TermWhipple procedure (pancreaticoduodenectomy)
- CancerHepatoblastoma
Shares Pancreatoblastoma, Cisplatin.
- TermCancer cachexia
- PathwayPancreatic cancer (KEGG map)