# Dhebri 2004: diagnosis, treatment and outcome of pancreatoblastoma

Source: https://onco.cc/key-papers/paper-dhebri-pancreatoblastoma-diagnosis-treatment-outcome-pancreatology-2004/  
OnCo record `paper-dhebri-pancreatoblastoma-diagnosis-treatment-outcome-pancreatology-2004` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

Complete 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.

## Fields

- Kind: Key paper
- Last checked: 2026-09-21
- Journal: Pancreatology
- Year: 2004
- DOI: 10.1159/000079823
- Authors: Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP.
- 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.
- What it means: 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.

## Sources

- Pancreatology 2004: https://doi.org/10.1159/000079823
- PubMed: https://pubmed.ncbi.nlm.nih.gov/15256806/

## Connected records

- cancers: [Pancreatic ductal adenocarcinoma](https://onco.cc/cancers/pancreatic/), [Pancreatoblastoma](https://onco.cc/cancers/pancreatoblastoma/)

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