{"entity":{"id":"paper-dhebri-pancreatoblastoma-diagnosis-treatment-outcome-pancreatology-2004","kind":"paper","name":"Dhebri 2004: diagnosis, treatment and outcome of pancreatoblastoma","aka":[],"tldr":"A review pooling every published case of pancreatoblastoma to that date, showing that it is mainly a disease of young children, that alpha-fetoprotein is a useful marker, that surgery is the treatment that cures, and that adults do worse than children.","summary":"Systematic review of pancreatoblastoma cases reported in the literature, summarising age and sex distribution, presenting features, imaging, the role of alpha-fetoprotein, histology, treatment and outcome. Most cases were in children under ten, with a smaller adult group; tumours were usually large, and metastases at diagnosis were common.\n\nComplete resection was associated with long-term survival in children, chemotherapy produced responses in unresectable disease, and radiotherapy had a limited role. Adult patients had a markedly worse prognosis, with most dying of disease.","asOf":"2026-09-21","links":[{"label":"Pancreatology 2004","url":"https://doi.org/10.1159/000079823"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/15256806/"}],"tags":[],"related":[],"cancers":["pancreatoblastoma","pancreatic"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"journal":"Pancreatology","year":2004,"doi":"10.1159/000079823","pmid":"15256806","authors":"Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP.","paperType":"review","findings":["Pancreatoblastoma is predominantly a childhood tumour; adults form a minority with a poorer outcome.","Alpha-fetoprotein is raised in most children and tracks response.","Complete resection is the treatment associated with cure; chemotherapy helps unresectable disease."],"whatItMeans":"The review is the usual citation for the natural history of pancreatoblastoma and for the recommendation of resection with chemotherapy for advanced disease.","caveats":["Literature-based review with publication bias and heterogeneous reporting.","Predates the EXPeRT series and modern chemotherapy protocols."]},"route":"/key-papers/paper-dhebri-pancreatoblastoma-diagnosis-treatment-outcome-pancreatology-2004/","neighbours":{"cancer":[{"id":"pancreatic","kind":"cancer","name":"Pancreatic ductal adenocarcinoma","route":"/cancers/pancreatic/"},{"id":"pancreatoblastoma","kind":"cancer","name":"Pancreatoblastoma","route":"/cancers/pancreatoblastoma/"}]}}