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9 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.
Hepatoblastoma is a childhood liver cancer, mostly of toddlers, cured in most standard-risk cases with cisplatin chemotherapy and surgery, including liver transplant when the tumour cannot be cut out. Sodium thiosulfate given after cisplatin halves the permanent hearing loss cisplatin causes, and became the first approved otoprotectant in 2022.
Hepatoblastoma is an embryonal liver tumour with CTNNB1 (β-catenin) mutations in most cases, associated with prematurity, Beckwith-Wiedemann syndrome and familial adenomatous polyposis. Alpha-fetoprotein (AFP) is elevated in >90% and tracks response; very low AFP (<100 ng/mL) marks an aggressive small-cell-undifferentiated variant. Staging uses PRETEXT (extent within the liver) plus annotation factors (vascular involvement, extrahepatic extension, metastases, rupture) under the international CHIC risk stratification.
Treatment combines cisplatin-based chemotherapy with complete surgical resection; standard-risk disease is cured in ~90% with cisplatin monotherapy (SIOPEL-3), high-risk disease uses cisplatin-doxorubicin (PLADO) or dose-dense cisplatin (SIOPEL-4), and unresectable tumours confined to the liver are transplanted with excellent outcomes. The Paediatric Hepatic International Tumour Trial (PHITT) harmonises COG, SIOPEL and JPLT approaches. Sodium thiosulfate given 6 hours after cisplatin halves permanent hearing loss without compromising survival (SIOPEL-6, NEJM 2018), leading to FDA approval of the first otoprotectant (2022).
| Setting | Approach | Guideline |
|---|---|---|
| Very low / standard risk (PRETEXT I-III, resectable, AFP >100) | Upfront resection for PRETEXT I-II (COG) or cisplatin monotherapy ×4-6 with delayed resection (SIOPEL-3); sodium thiosulfate after each cisplatin dose for otoprotection. | not mapped |
| High risk (metastatic, AFP <100, PRETEXT IV, vascular involvement) | Cisplatin-doxorubicin (PLADO) or dose-dense cisplatin (SIOPEL-4), resection of primary and lung metastases; consider C5VD (COG). | not mapped |
| Unresectable after chemotherapy (POST-TEXT IV, central vascular involvement) | Orthotopic liver transplantation (5-year survival ~80%); early referral to a transplant centre. | not mapped |
| Relapsed/refractory | Irinotecan-based salvage, surgery for isolated recurrence, transplant if liver-confined; trials. | not mapped |