Hepatoblastoma

Section editor
	Wayne H. Liang, MD, MS UAB Birmingham, AL LinkedIn WayneLiangMD

7 regimens on this page 7 variants on this page

All lines of therapy

Cisplatin monotherapy

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Regimen

Study	Evidence	Comparator	Comparative Efficacy
Perilongo et al. 2009 (SIOPEL 3)	Phase III (E-de-esc)	Cisplatin & Doxorubicin	Non-inferior complete resection rate

Chemotherapy

• Cisplatin (Platinol)

References

1. SIOPEL 3: Perilongo G, Maibach R, Shafford E, Brugieres L, Brock P, Morland B, de Camargo B, Zsiros J, Roebuck D, Zimmermann A, Aronson D, Childs M, Widing E, Laithier V, Plaschkes J, Pritchard J, Scopinaro M, MacKinlay G, Czauderna P. Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma. N Engl J Med. 2009 Oct 22;361(17):1662-70. link to original article PubMed

Cisplatin & Doxorubicin

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PLADO: PLAtinol (Cisplatin) & DOxorubicin

Regimen

Study	Evidence	Comparator	Comparative Efficacy	Comparative Toxicity
Ortega et al. 2000	Phase III (E-switch-ic)	Cisplatin, 5-FU, Vincristine		More toxic
Perilongo et al. 2009 (SIOPEL 3)	Phase III (C)	Cisplatin	Non-inferior complete resection rate

Chemotherapy

• Cisplatin (Platinol)
• Doxorubicin (Adriamycin)

References

1. Ortega JA, Douglass EC, Feusner JH, Reynolds M, Quinn JJ, Finegold MJ, Haas JE, King DR, Liu-Mares W, Sensel MG, Krailo MD. Randomized comparison of cisplatin/vincristine/fluorouracil and cisplatin/continuous infusion doxorubicin for treatment of pediatric hepatoblastoma: a report from the Children's Cancer Group and the Pediatric Oncology Group. J Clin Oncol. 2000 Jul;18(14):2665-75. link to original article PubMed
2. SIOPEL 3: Perilongo G, Maibach R, Shafford E, Brugieres L, Brock P, Morland B, de Camargo B, Zsiros J, Roebuck D, Zimmermann A, Aronson D, Childs M, Widing E, Laithier V, Plaschkes J, Pritchard J, Scopinaro M, MacKinlay G, Czauderna P. Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma. N Engl J Med. 2009 Oct 22;361(17):1662-70. link to original article PubMed

Cisplatin, Fluorouracil, Vincristine

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Regimen

Study	Evidence	Comparator	Comparative Efficacy
Katzenstein et al. 2019 (AHEP0731)	Phase III (E-esc)	Observation	Seems to have superior EFS

For stage 1 or 2 hepatoblastoma without 100% pure fetal stage I or small-cell undifferentiated histology with complete resection at diagnosis and no history of prior chemotherapy or liver directed therapy. Eligible patients include: patients< 21 years old, elevated serum α-fetoprotein level (>100 ng/mL), at least 50% Karnofsky (patients >16 years) or Lansky (patients ≤16 years) performance status.

Preceding treatment

• Curative surgical resection

Chemotherapy

• Cisplatin as follows:
  • Less than 10 kg: 3.3 mg/kg IV over 6 hours once on day 1
  • 10 kg or more: 100 mg/m² over 6 hours once on day 1
• Fluorouracil as follows:
  • Less than 10 kg: 20 mg/kg IV once on day 2
  • 10 kg or more: 600 mg/m² IV once on day 2
• Vincristine as follows:
  • Less than 10 kg: 0.05 mg/kg IV once per day on days 2, 9, 16
  • 10 kg or more: 1.5 mg/m² (maximum dose of 2 mg) IV once per day on days 2, 9, 16

21-day cycle for 1 cycle (to be given within 42 days of resection)

References

1. AHEP0731: Katzenstein HM, Langham MR, Malogolowkin MH, Krailo MD, Towbin AJ, McCarville MB, Finegold MJ, Ranganathan S, Dunn S, McGahren ED, Tiao GM, O'Neill AF, Qayed M, Furman WL, Xia C, Rodriguez-Galindo C, Meyers RL. Minimal adjuvant chemotherapy for children with hepatoblastoma resected at diagnosis (AHEP0731): a Children's Oncology Group, multicentre, phase 3 trial. Lancet Oncology. 2019 May 1. 20(5) 719-727. Epub 2019 Apr 8. link to original article PubMed