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The defining description of pancreatoblastoma, the rare pancreatic cancer of young children: its distinctive mix of enzyme-producing cells and whorled squamoid nests under the microscope, its generally good outlook after complete removal in children, and its more aggressive behaviour in adults.
Clinicopathological study of pancreatoblastomas from the Armed Forces Institute of Pathology with a review of the published literature. The tumours were large and mostly arose in the first decade of life, though adult cases occurred; some were associated with Beckwith-Wiedemann syndrome and many produced alpha-fetoprotein.
Histologically the tumours combined acinar differentiation (confirmed by trypsin, chymotrypsin and lipase immunostains) with squamoid nests, and variable neuroendocrine and ductal elements, distinguishing them from acinar cell carcinoma. Complete resection was associated with cure in most children, while adults and patients with metastases fared poorly.
Pathologists diagnose pancreatoblastoma by the criteria set out here, and the paper established that the paediatric and adult forms behave differently.