Pediatric Surgery International

, Volume 23, Issue 2, pp 103–109 | Cite as

Treatment outcomes for hepatoblastoma: an institution’s experience over two decades

  • J. P. AngEmail author
  • J. A. Heath
  • S. Donath
  • S. Khurana
  • A. Auldist
Original Article


Our objective is to review a leading Australian tertiary referral centre’s experience in treating hepatoblastoma over two decades. This is a retrospective study of clinical, radiological and pathological data in hepatoblastoma patients treated at our institution between 1984 and 2004. Thirty children (17 male, 13 female) ranging in age from 5 months to 6.5 years (median 19 months) at diagnosis were reviewed. This is the largest case series reported in Australia to date. The median length of follow-up was 6.3 years (range 7 months to 20.9 years). On average, the number of new cases increased by 0.12 each year (P = 0.01). A total of 29 patients underwent definitive surgery for primary tumour control. Of these, two (7%) had initial primary surgery, while 27 (93%) had delayed surgery following neo-adjuvant chemotherapy. Chemotherapy regimens included the SIOPEL study protocols. Patients not enrolled in SIOPEL tended to be given more courses of chemotherapy. Ten patients (34%) received an extended right hemi-hepatectomy, six (21%) had right hemi-hepatectomy, seven (24%) had left hemi-hepatectomy, three (10%) had left lateral segmentectomy, and two (7%) had a non-anatomical resection. In addition, two patients required IVC reconstruction at the time of their primary liver resection. Overall survival at 5 years was 89.1% (95% CI 69.8–96.4%). Event-free survival at 5 years was 75.7% (95% CI 53.2–88.5%). There was a clear increase in the risk of recurrence (RR = 4.8) and death (RR = 4.5) where margins were not microscopically clear. However, neither reached statistical significance in this small cohort. Surgery was still worthwhile even when margins were involved. There was no correlation between mean AFP level at diagnosis, and outcome. Our experience suggests that the achievement of microscopically clear margins at the time of primary tumour resection is an important factor in achieving a positive long-term outcome. In addition, extended courses of neo-adjuvant chemotherapy in patients with pulmonary metastases achieves good long-term outcomes, provided adequate surgical resection of the primary tumour with clear microscopic margins can be achieved.


Hepatoblastoma Treatment Outcome Liver resection Survival 



Many thanks to Vicky Thursfield of Cancer Council Victoria; Juan-Carlos Patillo and Tom Clarnette of the Department of Surgery (RCH); and the data managers of the Children’s Cancer Centre.


  1. 1.
    Raney B (1997) Hepatoblastoma in children: a review. J Pediatr Hematol Oncol 19(5):418–422PubMedCrossRefGoogle Scholar
  2. 2.
    Perilongo G, Shafford E, Plaschkes J (2000) SIOPEL trials using preoperative chemotherapy in hepatoblastoma. Lancet Oncol 1:94–100PubMedCrossRefGoogle Scholar
  3. 3.
    Schnater JM, Kohler SE, Lamers WH, von Schweinitz D, Aronson DC (2003) Where do we stand with hepatoblastoma? A review. Cancer 98(4):668–678PubMedCrossRefGoogle Scholar
  4. 4.
    Ansell P, Mitchell CD, Roman E, Simpson J, Birch JM, Eden TOB (2005) Relationships between perinatal and maternal characteristics and hepatoblastoma: a report from the UKCCS. Eur J Cancer 41(5):741–748PubMedCrossRefGoogle Scholar
  5. 5.
    Feusner J, Plaschkes J (2002) Hepatoblastoma and low birth weight: a trend or chance observation? Med Pediatr Oncol 39(5):508–509PubMedCrossRefGoogle Scholar
  6. 6.
    Ikeda H, Hachitanda Y, Tanimura M, Maruyama K, Koizumi T, Tsuchida Y (1998) Development of unfavorable hepatoblastoma in children of very low birth weight: results of a surgical and pathologic review. Cancer 82(9):1789–1796PubMedCrossRefGoogle Scholar
  7. 7.
    Ikeda H, Matsuyama S, Tanimura M (1997) Association between hepatoblastoma and very low birth weight: a trend or a chance? J Pediatr 130(4):557–560PubMedCrossRefGoogle Scholar
  8. 8.
    Kapfer SA, Petruzzi MJ, Caty MG (2004) Hepatoblastoma in low birth weight infants: an institutional review. Pediatr Surg Int 20(10):753–756PubMedCrossRefGoogle Scholar
  9. 9.
    Maruyama K, Ikeda H, Koizumi T, Tsuchida Y (1999) Prenatal and postnatal histories of very low birthweight infants who developed hepatoblastoma. Pediatr Int 41(1):82–89PubMedCrossRefGoogle Scholar
  10. 10.
    Maruyama K, Ikeda H, Koizumi T, Tsuchida Y, Tanimura M, Nishida H, Takahashi N, Fujimura M, Tokunaga Y (2000) Case-control study of perinatal factors and hepatoblastoma in children with an extremely low birthweight. Pediatr Int 42(5):492–498PubMedCrossRefGoogle Scholar
  11. 11.
    Oue T, Kubota A, Okuyama H, Kawahara H, Nara K, Kawa K, Kitajima H (2003) Hepatoblastoma in children of extremely low birth weight: a report from a single perinatal center. J Pediatr Surg 38(1):134–137PubMedCrossRefGoogle Scholar
  12. 12.
    Brown J, Perilongo G, Shafford E, Keeling J, Pritchard J, Brock P, Dicks-Mireaux C, Phillips A, Vos A, Plaschkes J (2000) Pretreatment prognostic factors for children with hepatoblastoma—results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1. Eur J Cancer 36(11):1418–1425PubMedCrossRefGoogle Scholar
  13. 13.
    Douglass EC, Reynolds M, Finegold M, Cantor AB, Glicksman A (1993) Cisplatin, vincristine, and fluorouracil therapy for hepatoblastoma: a Pediatric Oncology Group study. J Clin Oncol 11(1):96–99PubMedGoogle Scholar
  14. 14.
    Ehrlich PF, Greenberg ML, Filler RM (1997) Improved long-term survival with preoperative chemotherapy for hepatoblastoma. J Pediatr Surg 32(7):999–1003PubMedCrossRefGoogle Scholar
  15. 15.
    Fuchs J, Bode U, von Schweinitz D, Weinel P, Erttmann R, Harms D, Mildenberger H (1999) Analysis of treatment efficiency of carboplatin and etoposide in combination with radical surgery in advanced and recurrent childhood hepatoblastoma: a report of the German Cooperative Pediatric Liver Tumor Study HB 89 and HB 94. Klin Padiatr 211(4):305–309PubMedCrossRefGoogle Scholar
  16. 16.
    Fuchs J, Rydzynski J, Von Schweinitz D, Bode U, Hecker H, Weinel P, Burger D, Harms D, Erttmann R, Oldhafer K, Mildenberger H (2002) Pretreatment prognostic factors and treatment results in children with hepatoblastoma: a report from the German Cooperative Pediatric Liver Tumor Study HB 94. Cancer 95(1):172–182PubMedCrossRefGoogle Scholar
  17. 17.
    Ortega JA, Douglass EC, Feusner JH, Reynolds M, Quinn JJ, Finegold MJ, Haas JE, King DR, Liu-Mares W, Sensel MG, Krailo MD (2000) 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 18(14):2665–2775PubMedGoogle Scholar
  18. 18.
    Perilongo G, Brown J, Shafford E, Brock P, De Camargo B, Keeling JW, Vos A, Philips A, Pritchard J, Plaschkes J (2000) Hepatoblastoma presenting with lung metastases: treatment results of the first cooperative, prospective study of the International Society of Paediatric Oncology on childhood liver tumors. Cancer 89(8):1845–1853PubMedCrossRefGoogle Scholar
  19. 19.
    Perilongo G, Shafford E, Maibach R, Aronson D, Brugieres L, Brock P, Childs M, Czauderna P, MacKinlay G, Otte JB, Pritchard J, Rondelli R, Scopinaro M, Staalman C, Plaschkes J (2004) Risk-adapted treatment for childhood hepatoblastoma. Final report of the second study of the International Society of Paediatric Oncology—SIOPEL 2. Eur J Cancer 40(3):411–421PubMedCrossRefGoogle Scholar
  20. 20.
    Stringer MD, Hennayake S, Howard ER, Spitz L, Shafford EA, Mieli-Vergani G, Saxena R, Malone M, Dicks-Mireaux C, Karani J (1995) Improved outcome for children with hepatoblastoma. Br J Surg 82(3):386–391PubMedGoogle Scholar
  21. 21.
    Suita S, Tajiri T, Takamatsu H, Mizote H, Nagasaki A, Inomata Y, Hara T, Okamura J, Miyazaki S, Kawakami K, Eguchi H, Tsuneyoshi M (2004) Improved survival outcome for hepatoblastoma based on an optimal chemotherapeutic regimen—a report from the study group for pediatric solid malignant tumors in the Kyushu area. J Pediatr Surg 39(2):195–198PubMedCrossRefGoogle Scholar
  22. 22.
    von Schweinitz D (2000) Identification of risk groups in hepatoblastoma—another step in optimising therapy. Eur J Cancer 36(11):1343–1346CrossRefGoogle Scholar
  23. 23.
    von Schweinitz D, Byrd DJ, Hecker H, Weinel P, Bode U, Burger D, Erttmann R, Harms D, Mildenberger H (1997) Efficiency and toxicity of ifosfamide, cisplatin and doxorubicin in the treatment of childhood hepatoblastoma. Eur J Cancer 33(8):1243–1249CrossRefGoogle Scholar
  24. 24.
    Brugieres L, Plaschkes J, MacKinlay G, Cecchetto P, Czauderna P, Aronson D, Zimmerman A, Perilongo G (2000) Hepatoblastoma—microscopical residual disease (MRD) after delayed surgery; prognostic implications. Med Pediatr Oncol 35(3):177Google Scholar
  25. 25.
    Towu E, Kiely E, Pierro A, Spitz L (2004) Outcome and complications after resection of hepatoblastoma. J Pediatr Surg 39(2):199–202PubMedCrossRefGoogle Scholar
  26. 26.
    Dicken BJ, Bigam DL, Lees GM (2004) Association between surgical margins and long-term outcome in advanced hepatoblastoma. J Pediatr Surg 39(5):721–725PubMedCrossRefGoogle Scholar
  27. 27.
    Schnater JM, Aronson DC, Plaschkes J, Perilongo G, Brown J, Otte J-B, Brugieres L, Czauderna P, MacKinlay G, Vos A (2002) Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group. Cancer 94(4):1111–1120PubMedCrossRefGoogle Scholar
  28. 28.
    Otte JB, de Ville de Goyet J, Reding R (2005) Liver transplantation for hepatoblastoma: indications and contraindications in the modern era. Pediatr Transplant 9(5):557–565PubMedCrossRefGoogle Scholar
  29. 29.
    Otte JB, Pritchard J, Aronson DC, Brown J, Czauderna P, Maibach R, Perilongo G, Shafford E, Plaschkes J (2004) Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience. Pediatr Blood Cancer 42(1):74–83PubMedCrossRefGoogle Scholar
  30. 30.
    Czauderna P, Otte JB, Aronson DC, Gauthier F, Mackinlay G, Roebuck D, Plaschkes J, Perilongo G (2005) Guidelines for surgical treatment of hepatoblastoma in the modern era—recommendations from the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL). Eur J Cancer 41(7):1031–1036PubMedCrossRefGoogle Scholar
  31. 31.
    Austin MT, Leys CM, Feurer ID, Lovvorn HN III, O’Neill JA Jr, Pinson CW, Pietsch JB (2006) Liver transplantation for childhood hepatic malignancy: a review of the United Network for Organ Sharing (UNOS) database. J Pediatr Surg 41(1):182–186PubMedCrossRefGoogle Scholar
  32. 32.
    Tiao GM, Bobey N, Allen S, Nieves N, Alonso M, Bucuvalas J, Wells R, Ryckman F (2005) The current management of hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr 146(2):204–211PubMedCrossRefGoogle Scholar
  33. 33.
    Ortega JA, Krailo MD, Haas JE, King DR, Ablin AR, Quinn JJ, Feusner J, Campbell JR, Lloyd DA, Cherlow J (1991) Effective treatment of unresectable or metastatic hepatoblastoma with cisplatin and continuous infusion doxorubicin chemotherapy: a report from the Childrens Cancer Study Group. J Clin Oncol 9(12):2167–2176PubMedGoogle Scholar
  34. 34.
    Van Tornout JM, Buckley JD, Quinn JJ, Feusner JH, Krailo MD, King DR, Hammond GD, Ortega JA (1997) Timing and magnitude of decline in alpha-fetoprotein levels in treated children with unresectable or metastatic hepatoblastoma are predictors of outcome: a report from the Children’s Cancer Group. J Clin Oncol 15(3):1190–1197PubMedGoogle Scholar
  35. 35.
    von Schweinitz D, Hecker H, Harms D, Bode U, Weinel P, Burger D, Erttmann R, Mildenberger H (1995) Complete resection before development of drug resistance is essential for survival from advanced hepatoblastoma—a report from the German Cooperative Pediatric Liver Tumor Study HB-89. J Pediatr Surg 30(6):845–852CrossRefGoogle Scholar
  36. 36.
    Reynolds P, Urayama KY, Von Behren J, Feusner J (2004) Birth characteristics and hepatoblastoma risk in young children. Cancer 100(5):1070–1076PubMedCrossRefGoogle Scholar
  37. 37.
    Ross JA, Gurney JG (1998) Hepatoblastoma incidence in the United States from 1973 to 1992. Med Pediatr Oncol 30(3):141–142PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • J. P. Ang
    • 1
    Email author
  • J. A. Heath
    • 2
  • S. Donath
    • 3
  • S. Khurana
    • 1
  • A. Auldist
    • 1
  1. 1.Department of SurgeryRoyal Children’s HospitalParkvilleAustralia
  2. 2.Children’s Cancer CentreRoyal Children’s HospitalParkvilleAustralia
  3. 3.Clinical Epidemiology and Biostatistics UnitRoyal Children’s HospitalParkvilleAustralia

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