Definition:
- Primary malignant liver tumor arising from immature liver precursor cells (hepatoblasts).
- Most common liver malignancy in children, usually <3 years old.
Epidemiology
- Rare: ~1–2 per million children annually
- Peak incidence: 6 months–3 years
- Risk factors: prematurity, very low birth weight, Beckwith-Wiedemann syndrome, familial adenomatous polyposis (FAP)
Clinical Features
- Abdominal mass – firm, often right upper quadrant
- Hepatomegaly
- Symptoms: anorexia, weight loss, vomiting, sometimes jaundice
- Lab: markedly elevated alpha-fetoprotein (AFP) in most cases
Staging & Risk
- PRETEXT system (Pretreatment Extent of Disease) guides treatment and surgical planning
- Risk stratification: standard-risk vs high-risk based on tumor size, PRETEXT stage, metastases, AFP level
Treatment & Pharmacist Role
1. Surgery
- Complete hepatectomy if feasible
- Liver transplant in unresectable tumors
2. Chemotherapy
- Cisplatin-based regimens are backbone
- Common regimens:
- Cisplatin alone → standard-risk
- Cisplatin + Doxorubicin → high-risk or advanced disease
3. Pharmacist Considerations
- Cisplatin: nephrotoxicity, ototoxicity → monitor renal function, audiometry, hydration
- Doxorubicin: cardiotoxicity → monitor cumulative dose, echocardiography
- Myelosuppression → monitor CBC, provide growth factor support if needed
- Pediatric dosing based on BSA or weight
High-Yield Pharmacist Pearls