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Childhood Liver Cancer Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Hepatoblastoma

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Treatment Options Under Clinical Evaluation

The following are examples of national and/or institutional clinical trials that are currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

Stages I and II

  • COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer): Attempted complete surgical resection for all PRETEXT 1 tumors and those PRETEXT 2 tumors with greater than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, or the portal bifurcation. Biopsy only of PRETEXT 2 tumors with less than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, and the portal bifurcation.
    • Stage I pure fetal histology with an alpha-fetoprotein (AFP) level greater than 100 ng/ml, non-small cell undifferentiated (very low risk): Resection and observation with no chemotherapy.
    • Stage I and II with an AFP level greater than 100 ng/ml, non-small cell undifferentiated (low risk): Cisplatin/5-fluorouracil/vincristine for two 3-week cycles.
    • Stage I and II with an AFP level greater than 100 ng/ml with small cell undifferentiated elements (intermediate risk): Cisplatin/5-fluorouracil/vincristine/doxorubicin for six cycles, with dexrazoxane the last two cycles.

Stage III

The following treatment option is under investigation in a COG clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

  • COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer):
    • Biopsy only of PRETEXT 2 tumors with less than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, and the portal bifurcation, and of PRETEXT 3 and PRETEXT 4 tumors.
    • Gross residual disease/unresected disease non-small cell undifferentiated, AFP level greater than 100 ng/ml (intermediate risk): Two to four cycles of cisplatin/5-fluorouracil/vincristine/doxorubicin (C5VD) followed by resection or orthotopic liver transplant, then an additional two to four cycles of C5VD to total six cycles of chemotherapy, the last two cycles including dexrazoxane.
    • Use of the PRETEXT staging system after chemotherapy to determine resectability (termed POSTTEXT): Tumors considered resectable after two or four cycles of chemotherapy:
      • Tumors with POSTTEXT 1.
      • Tumor with POSTTEXT 2 with greater than 1 cm radiographic margin on the middle hepatic vein, the retrohepatic inferior vena cava, or the portal bifurcation.
    • Stage III tumors with an AFP level less than 100 ng/ml (high risk).
    • Patients with primary hepatoblastomas that remain unresectable, defined as tumors with less than 1 cm radiographic venous margins, POSTTEXT 3 multifocal or POSTTEXT 4, will be referred to a liver transplant center after the first two cycles of C5VD. Resection or orthotopic liver transplant will take place after four cycles of C5VD.

Stage IV

The following treatment option is under investigation in a COG clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

  • COG-AHEP0731 (Combination Chemotherapy in Treating Young Patients With Newly Diagnosed Liver Cancer):
    • Stage IV tumors with any histology and any AFP level or any stage tumor with an AFP level less than 100 ng/ml (high risk): The treatment regimen includes two cycles of vincristine/irinotecan (VI) followed by six cycles of C5VD. Those whose tumor responds to VI will also receive two additional cycles of VI, totaling ten cycles of chemotherapy. Resection to be determined by POSTTEXT staging as above.
    • On this study, residual metastases are resected at the time of definitive surgery or, in the case of liver transplant, prior to transplantation to render the patient free of extrahepatic disease prior to transplant. Those who cannot be rendered free of extrahepatic disease will continue chemotherapy and not undergo transplant.

References:

  1. Malogolowkin MH, Katzenstein HM, Meyers RL, et al.: Complete surgical resection is curative for children with hepatoblastoma with pure fetal histology: a report from the Children's Oncology Group. J Clin Oncol 29 (24): 3301-6, 2011.
  2. Haas JE, Feusner JH, Finegold MJ: Small cell undifferentiated histology in hepatoblastoma may be unfavorable. Cancer 92 (12): 3130-4, 2001.
  3. Douglass EC, Reynolds M, Finegold M, et al.: Cisplatin, vincristine, and fluorouracil therapy for hepatoblastoma: a Pediatric Oncology Group study. J Clin Oncol 11 (1): 96-9, 1993.
  4. Perilongo G, Shafford E, Maibach R, et al.: 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): 411-21, 2004.
  5. Pritchard J, Brown J, Shafford E, et al.: Cisplatin, doxorubicin, and delayed surgery for childhood hepatoblastoma: a successful approach--results of the first prospective study of the International Society of Pediatric Oncology. J Clin Oncol 18 (22): 3819-28, 2000.
  6. Ortega JA, Douglass EC, Feusner JH, et al.: 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): 2665-75, 2000.
  7. Ortega JA, Krailo MD, Haas JE, et al.: 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): 2167-76, 1991.
  8. Reynolds M, Douglass EC, Finegold M, et al.: Chemotherapy can convert unresectable hepatoblastoma. J Pediatr Surg 27 (8): 1080-3; discussion 1083-4, 1992.
  9. von Schweinitz D, Hecker H, Harms D, et al.: 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): 845-52, 1995.
  10. Malogolowkin MH, Katzenstein H, Krailo MD, et al.: Intensified platinum therapy is an ineffective strategy for improving outcome in pediatric patients with advanced hepatoblastoma. J Clin Oncol 24 (18): 2879-84, 2006.
  11. Reyes JD, Carr B, Dvorchik I, et al.: Liver transplantation and chemotherapy for hepatoblastoma and hepatocellular cancer in childhood and adolescence. J Pediatr 136 (6): 795-804, 2000.
  12. Otte JB, Pritchard J, Aronson DC, et al.: 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): 74-83, 2004.
  13. Molmenti EP, Wilkinson K, Molmenti H, et al.: Treatment of unresectable hepatoblastoma with liver transplantation in the pediatric population. Am J Transplant 2 (6): 535-8, 2002.
  14. Czauderna P, Otte JB, Aronson DC, et al.: 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): 1031-6, 2005.
  15. Austin MT, Leys CM, Feurer ID, et al.: Liver transplantation for childhood hepatic malignancy: a review of the United Network for Organ Sharing (UNOS) database. J Pediatr Surg 41 (1): 182-6, 2006.
  16. D'Antiga L, Vallortigara F, Cillo U, et al.: Features predicting unresectability in hepatoblastoma. Cancer 110 (5): 1050-8, 2007.
  17. Xianliang H, Jianhong L, Xuewu J, et al.: Cure of hepatoblastoma with transcatheter arterial chemoembolization. J Pediatr Hematol Oncol 26 (1): 60-3, 2004.
  18. Malogolowkin MH, Stanley P, Steele DA, et al.: Feasibility and toxicity of chemoembolization for children with liver tumors. J Clin Oncol 18 (6): 1279-84, 2000.
  19. Perilongo G, Brown J, Shafford E, et al.: 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): 1845-53, 2000.
  20. Zsíros J, Maibach R, Shafford E, et al.: Successful treatment of childhood high-risk hepatoblastoma with dose-intensive multiagent chemotherapy and surgery: final results of the SIOPEL-3HR study. J Clin Oncol 28 (15): 2584-90, 2010.
  21. Meyers RL, Katzenstein HM, Krailo M, et al.: Surgical resection of pulmonary metastatic lesions in children with hepatoblastoma. J Pediatr Surg 42 (12): 2050-6, 2007.
  22. Katzenstein HM, Rigsby C, Shaw PH, et al.: Novel therapeutic approaches in the treatment of children with hepatoblastoma. J Pediatr Hematol Oncol 24 (9): 751-5, 2002.
  23. Palmer RD, Williams DM: Dramatic response of multiply relapsed hepatoblastoma to irinotecan (CPT-11). Med Pediatr Oncol 41 (1): 78-80, 2003.
  24. Qayed M, Powell C, Morgan ER, et al.: Irinotecan as maintenance therapy in high-risk hepatoblastoma. Pediatr Blood Cancer 54 (5): 761-3, 2010.
  25. Habrand JL, Nehme D, Kalifa C, et al.: Is there a place for radiation therapy in the management of hepatoblastomas and hepatocellular carcinomas in children? Int J Radiat Oncol Biol Phys 23 (3): 525-31, 1992.
  26. Sue K, Ikeda K, Nakagawara A, et al.: Intrahepatic arterial injections of cisplatin-phosphatidylcholine-Lipiodol suspension in two unresectable hepatoblastoma cases. Med Pediatr Oncol 17 (6): 496-500, 1989.
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WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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