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8 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Pancreatoblastoma is the pancreatic cancer of young children, a tumour of immature pancreatic cells that behaves quite unlike adult pancreatic cancer. It grows as a large abdominal mass, often raises the blood marker alpha-fetoprotein, and is treated like the childhood liver cancer hepatoblastoma: chemotherapy to shrink it, then surgery, which cures most children whose tumour has not spread.
Pancreatoblastoma is an embryonal tumour that recapitulates fetal pancreatic development, with acinar, ductal and neuroendocrine differentiation and the squamoid nests that define it under the microscope. It presents in preschool children as a large, painless abdominal mass, sometimes with weight loss, vomiting or jaundice, and serum alpha-fetoprotein is raised in most. Alterations in the Wnt pathway (CTNNB1 mutations or APC loss) and loss of heterozygosity at 11p, the Beckwith-Wiedemann locus, are the recurrent genetic findings, and the tumour occurs in children with Beckwith-Wiedemann syndrome and in families with familial adenomatous polyposis. Adult cases occur and behave more aggressively.
Because the disease is so rare, treatment follows consensus from the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) and national rare-tumour registries rather than trials. Complete surgical resection is the cornerstone, usually a pancreatoduodenectomy or distal pancreatectomy; for the large tumours that cannot be removed at diagnosis, neoadjuvant chemotherapy with cisplatin and doxorubicin (the PLADO regimen used in hepatoblastoma) shrinks the tumour and makes surgery possible, and alpha-fetoprotein is followed as a response marker. Adjuvant chemotherapy is given after incomplete resection or for metastatic disease, and radiotherapy is reserved for residual disease. Metastases, most often to the liver, occur in a minority at diagnosis and are treated with chemotherapy and resection where possible.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis and staging | Imaging of abdomen and chest, serum alpha-fetoprotein and biopsy; genetic review for Beckwith-Wiedemann syndrome and familial adenomatous polyposis. | not mapped |
| Resectable at diagnosis | Complete resection (pancreatoduodenectomy or distal pancreatectomy); adjuvant chemotherapy after incomplete resection. | not mapped |
| Unresectable at diagnosis | Neoadjuvant cisplatin and doxorubicin (PLADO, as in hepatoblastoma) followed by delayed resection when the tumour shrinks. | not mapped |
| Metastatic or relapsed | Cisplatin and doxorubicin-based chemotherapy with resection of residual disease where possible; radiotherapy for unresectable residual tumour; international registry enrolment. | not mapped |
| Survivorship | Lifelong follow-up for pancreatic insufficiency, diabetes and the late effects of platinum and anthracycline chemotherapy. | not mapped |