# Hepatoblastoma > Hepatoblastoma for NEET-PG Paediatrics: AFP marker, low birth weight risk, PRETEXT staging, cisplatin chemotherapy and transplant for unresectable. - Canonical URL: https://prepelephant.com/topics/neet-pg/paediatrics/hepatoblastoma - Exam / course: NEET-PG · Subject: Paediatrics - Publisher: PrepElephant (https://prepelephant.com) — Prepared and reviewed by the PrepElephant Academic Review Team - First published: 2026-10-02 - Last updated: 2026-10-02 - How to cite: "Hepatoblastoma", PrepElephant, https://prepelephant.com/topics/neet-pg/paediatrics/hepatoblastoma ## Direct answer A painless right upper abdominal mass in a child under three years with a serum alpha-fetoprotein in the hundreds of thousands is hepatoblastoma — the commonest malignant liver tumour of early childhood, distinct from hepatocellular carcinoma, which favours children above five years with underlying liver disease or hepatitis B. Prematurity and very low birth weight are its strongest epidemiological associations, alongside Beckwith-Wiedemann syndrome, familial adenomatous polyposis and hemihyperplasia. Staging by the PRETEXT system (Pre-treatment EXTent of disease) divides the liver into four sections and grades I-IV by contiguous involvement, refined further by vascular invasion, extrahepatic spread and metastases — lungs first. Treatment is neoadjuvant cisplatin-based chemotherapy followed by complete surgical resection, with liver transplantation for tumours unresectable after chemotherapy; modern protocols cure more than eight in ten children. ## What you must remember - **Age and marker split:** hepatoblastoma peaks at 6 months to 3 years with alpha-fetoprotein elevated in roughly nine of ten; hepatocellular carcinoma favours older children, often on cirrhosis or hepatitis B. - **Risk associations:** very low birth weight and prematurity (risk rises steeply as birth weight falls — the viva favourite), Beckwith-Wiedemann syndrome, familial adenomatous polyposis and hemihyperplasia; such children enter surveillance with serial alpha-fetoprotein and ultrasound. - **PRETEXT in one line:** the liver divides into four sections; the number of contiguous tumour-free sections defines groups I-IV, with annotation factors (vascular invasion, extrahepatic spread, multifocality, rupture, nodes, metastases) refining risk. - **Chemotherapy backbone:** cisplatin monotherapy for standard risk, or cisplatin with doxorubicin, 5-fluorouracil and vincristine for higher risk; carboplatin regimens serve heart- or kidney-sparing strategies. - **Surgery is the cure:** complete resection — segmentectomy or hepatic lobectomy — after neoadjuvant shrinkage; PRETEXT I and II are often primary resections, III and IV go chemotherapy-first. - **Transplant role:** centrally placed or POSTTEXT III/IV tumours confined to the liver after chemotherapy, or those with major vascular invasion but no distant spread, are cured by transplantation in a majority. - **Follow-up anchors:** serial alpha-fetoprotein mirrors disease (half-life about five to seven days post-resection), chest imaging for pulmonary metastases (resectable metastases do not preclude cure), and audiometry with renal surveillance after cisplatin. - **Differential cluster:** Wilms tumour (renal, alpha-fetoprotein normal), neuroblastoma (suprarenal, calcification, urinary catecholamines), mesenchymal hamartoma (benign, cystic) and hepatocellular carcinoma. ## A staging-to-treatment walkthrough Take a 14-month-old, ex-preterm at 900 grams, with a nontender right upper quadrant mass found by the mother at bathtime; the child is active, mildly anaemic, liver span 9 cm, no jaundice. Step one, confirm organ and marker: ultrasound shows a solid right-lobe intrahepatic mass; alpha-fetoprotein is massively raised (age-adjusted — a newborn's normal is itself in the tens of thousands). Step two, stage: triple-phase CT or MRI with vascular reconstruction maps the sections involved — right anterior and posterior with a sliver of medial, PRETEXT III without vascular annotation; chest CT clears the lungs. Step three, risk-group and treat: PRETEXT III means cisplatin-based chemotherapy first, with interim alpha-fetoprotein tracking response (a failing marker triggers re-imaging). Step four, reassess resectability (POSTTEXT): the shrunken tumour leaves three free sections and a right hepatectomy follows; had it remained centrally unresectable, the child would be listed for living-donor transplantation, which Indian centres increasingly perform for exactly this indication. Step five, complete adjuvant chemotherapy, then alpha-fetoprotein and imaging on a tapering schedule, with audiometry and renal function for platinum late effects. Five-year survival is over 80 per cent — a number worth quoting when a frightened family asks about survival. ## Where students slip Not every raised alpha-fetoprotein means hepatoblastoma — infancy has physiological elevation, and hepatocellular carcinoma and yolk-sac tumours raise it too; age under three plus massive alpha-fetoprotein is the exam combination. The second slip is missing the prematurity link, the single most distinctive epidemiological fact. Third, students quote staging without knowing it decides sequence: PRETEXT I-II move to surgery early, III-IV go neoadjuvant, and unresectable disease is a transplant question, not a palliation question. ## Frequently asked questions ### What is the strongest epidemiological risk factor for hepatoblastoma? Very low birth weight and prematurity, with risk rising as birth weight falls; Beckwith-Wiedemann syndrome and familial adenomatous polyposis are the syndromic associations. ### Which tumour marker is used, and what are its pitfalls? Serum alpha-fetoprotein, elevated in about 90 per cent of cases and used for diagnosis, response tracking and surveillance — but physiological infancy levels are high, and hepatocellular carcinoma and yolk-sac tumours also raise it. ### What is the PRETEXT system? A surgical staging system dividing the liver into four sections and assigning groups I-IV by the number of contiguous tumour-free sections, with annotations for vascular invasion, spread and multifocality. ### When is liver transplantation indicated? For tumours that remain unresectable after neoadjuvant chemotherapy but are confined to the liver (including major vascular invasion), with cure rates comparable to resection in experienced centres. ### How does hepatoblastoma differ from hepatocellular carcinoma in children? Younger age, no need for underlying liver disease, cisplatin chemosensitivity and far better prognosis; hepatocellular carcinoma resists chemotherapy and usually arises on cirrhosis or hepatitis B.