PRETEXT and CHIC risk groups (hepatoblastoma)

What is measured: the extent of a hepatoblastoma and its risk group. How: contrast CT or MRI of the liver read against PRETEXT (I to IV by the number of contiguous liver sections free of tumour) with annotation factors V (hepatic veins or inferior vena cava), P (portal vein), E (contiguous extrahepatic spread), F (multifocal), R (rupture), C (caudate), N (nodes) and M (distant metastases, on chest CT); repeated after neoadjuvant chemotherapy as POST-TEXT to plan resection or transplant. Serum alpha-fetoprotein at diagnosis (under 100 ng/mL marks the small-cell undifferentiated type and a poor outlook; very high levels are also unfavourable) and age (under 3, 3 to 7, 8 and over) complete the CHIC stratification into very low, low, intermediate and high risk, which the PHITT trial used. What a result changes: very low risk (small tumour resected at diagnosis) gets surgery alone or minimal cisplatin; low risk gets cisplatin monotherapy with surgery after two cycles; intermediate risk gets cisplatin-based combinations; high risk (metastases, low AFP, PRETEXT IV with annotation factors) gets dose-dense cisplatin with doxorubicin and early referral for transplant when the tumour cannot be resected. Sodium thiosulfate is added to protect hearing from cisplatin. Germline APC (familial adenomatous polyposis) and 11p15 (Beckwith-Wiedemann) testing are considered. Where it matters: hepatoblastoma.