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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.
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.
The review is the usual citation for the natural history of pancreatoblastoma and for the recommendation of resection with chemotherapy for advanced disease.