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Hepatoblastoma

Most common pediatric liver malignancy with PRETEXT-based risk stratification and cisplatin-centered multimodal therapy

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

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What is Hepatoblastoma?

Hepatoblastoma — embryonal malignancy of liver arising from primitive hepatocytes; most cases diagnosed before age 3 (90% before age 5); presents as palpable abdominal mass, often with very elevated AFP (10,000-1,000,000 ng/mL); about 5% have normal AFP and portend worse prognosis.

PRETEXT staging — Pre-Treatment Extent of Disease system classifies based on number of liver sections involved (I-IV) and additional factors (V: venous invasion, P: portal vein, E: extrahepatic contiguous, F: multifocal, R: rupture, C: caudate, M: distant metastases, N: nodes); determines resectability and need for transplant.

Histologic subtypes — pure fetal (favorable), mixed fetal/embryonal, macrotrabecular, small cell undifferentiated (unfavorable), teratoid, transitional liver cell tumor; beta-catenin (CTNNB1) activation common driver; molecular subtypes emerging.

Treatment paradigm — cisplatin-based chemotherapy (SIOPEL protocols) with PLADO (cisplatin, doxorubicin) or monotherapy cisplatin for standard risk; surgical resection critical (complete resection required for cure); liver transplantation for unresectable central tumors (PRETEXT III-IV with vascular involvement).

Symptoms

Abdominal mass (most common) — palpable right upper quadrant, may be visible distension
Abdominal distension, decreased appetite, weight loss, failure to thrive
Jaundice (less common — advanced disease with bile duct compression)
Precocious puberty (rare — hCG-secreting tumors)
Thrombocytosis (paraneoplastic — common)
Metastatic symptoms — cough/dyspnea (lung), bone pain (rare)

Risk Factors

Beckwith-Wiedemann syndrome (overgrowth, hemihypertrophy — requires AFP screening)
Familial adenomatous polyposis (FAP — APC mutation)
Prematurity and very low birth weight (<1500 g)
Trisomy 18
Glycogen storage disease type Ia
Prior TPN exposure (infants)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Infant or young child with palpable abdominal mass warrants urgent imaging (ultrasound first-line), AFP level, and pediatric surgical-oncology evaluation.
  • Known Beckwith-Wiedemann syndrome, FAP, or other predisposing condition requires regular AFP screening and abdominal ultrasound every 3 months until age 4-5.
  • Persistent failure to thrive, decreased appetite, or unexplained thrombocytosis in a young child warrants evaluation including AFP and abdominal imaging.

Treatment Methods

01
Diagnostic workup — abdominal ultrasound and contrast-enhanced MRI or CT (primary lesion and PRETEXT assessment), chest CT (lung metastases 10-20%), AFP level (tumor marker and monitoring), liver function, coagulation; biopsy needed except for straightforward PRETEXT I amenable to upfront resection with typical AFP.
02
Risk stratification — SIOPEL criteria: standard risk (PRETEXT I-III without adverse features) vs high risk (PRETEXT IV, VPEFR annotation positive, metastases, AFP <100, or age >5 years); SIOPEL-6 (cisplatin monotherapy) successful for standard risk reducing ototoxicity.
03
Chemotherapy — cisplatin monotherapy for standard risk; PLADO (cisplatin plus doxorubicin) or SUPERPLADO (cisplatin, doxorubicin, carboplatin) for high-risk or metastatic; sodium thiosulfate (STS) given 6h after cisplatin per SIOPEL-6 reduces hearing loss 50%.
04
Surgical resection — partial hepatectomy (segmentectomy, extended hepatectomy) for tumors confined to resectable sections after neoadjuvant chemotherapy; experienced pediatric hepatobiliary surgery team; complete R0 resection required for cure.
05
Liver transplantation — for unresectable PRETEXT III or IV with vascular involvement, or centrally located tumors; timing after maximal chemotherapy response and no extrahepatic disease; 5-year survival 75-85% for PRETEXT III-IV with transplant.
06
Metastatic and relapsed disease — intensified chemotherapy for metastatic (ICE or carboplatin/etoposide), pulmonary metastasectomy for residual lung disease, relapsed disease poorer prognosis requiring clinical trial enrollment (beta-catenin inhibitors, sorafenib in trials).

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.