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Liver Tumors

Childhood Liver Cancers

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Rare Tumors In Children and Adolescents

Part of the book series: Pediatric Oncology ((PEDIATRICO))

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Abstract

Liver “masses” are not uncommon and are linked with a wide assortment of conditions. Infections and fatty deposits can mimic a tumor. Other conditions, such as choledochal cysts and vascular malformations will also present as liver “masses”. In this chapter, we will confine our discussion to malignant liver tumors. Liver tumors account for about 1% of all pediatric cancers or approximately 2.4 cases/100,000. Each year in the USA, approximately 125–150 new cases of liver cancer are diagnosed in children (Ries et al., Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. Bethesda, National Cancer Institute, 1999). Hepatoblastoma is the most frequent malignant liver tumor in children and represents approximately two thirds of all pediatric liver cancers (Exelby et al., J Pediatr Surg 10(3):329–337, 1975). Hepatoblastoma usually arises in the first 2 years of life, with a mean age of onset of approximately 16–20 months, and almost all cases occur before 4 years of age (Darbari et al., Hepatology 38(3):560–566, 2003). The incidence of hepatoblastoma has been increasing over the last several decades (Ross and Gurney, Med Pediatr Oncol 30(3):141–142, 1998). Hepatoblastoma tumor cells resemble cells seen in the developing liver, and therefore, this tumor is categorized as an embryonal tumor. Hepatocellular carcinoma (HCC) is the main liver tumor manifesting in the second decade of life. Both hepatoblastoma and hepatocellular carcinoma seem to arise more commonly in boys than in girls. Other pathologically distinct liver tumors are less frequently diagnosed in children and include undifferentiated embryonal sarcoma, rhabdoid tumor, rhabdomyosarcoma, germ cell tumors, lymphoma, leiomyosarcoma, and angiosarcoma. Benign neoplasms account for approximately one third of all pediatric liver lesions and include vascular tumors (hemangiomas and hemangioendotheliomas), mesenchymal hamartomas, focal nodular hyperplasia, and adenomas.

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References

  • Abou-Alfa GK, Johnson P et al (2010) Doxorubicin plus sorafenib vs. doxorubicin alone in patients with advanced hepatocellular carcinoma: a randomized trial. JAMA 304(19):2154–2160

    Article  PubMed  CAS  Google Scholar 

  • Aronson DC, Schnater JM et al (2005) Predictive value of the pretreatment extent of disease system in hepatoblastoma: results from the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL-1 study. J Clin Oncol 23(6):1245–1252

    Article  PubMed  Google Scholar 

  • Baron PW, Majlessipour F et al (2007) Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection. J Gastrointest Surg 11(1):73–75

    Article  PubMed  Google Scholar 

  • Beaty O 3rd, Berg S et al (2010) A phase II trial and pharmacokinetic study of oxaliplatin in children with refractory solid tumors: a Children’s Oncology Group study. Pediatr Blood Cancer 55(3):440–445

    Article  PubMed  Google Scholar 

  • Blohm ME, Vesterling-Horner D et al (1998) Alpha 1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Oncol 15(2):135–142

    Article  PubMed  CAS  Google Scholar 

  • Buckley JD, Sather H et al (1989) A case-control study of risk factors for hepatoblastoma. A report from the Childrens Cancer Study Group. Cancer 64(5):1169–1176

    Article  PubMed  CAS  Google Scholar 

  • Chang MH, Chen CJ et al (1997) Universal hepatitis B vaccination in Taiwan and the incidence of hepatocellular carcinoma in children. Taiwan Childhood Hepatoma Study Group. N Engl J Med 336(26):1855–1859

    Article  PubMed  CAS  Google Scholar 

  • Clericuzio CL, Chen E et al (2003) Serum alpha-fetoprotein screening for hepatoblastoma in children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia. J Pediatr 143(2):270–272

    Article  PubMed  Google Scholar 

  • Czauderna P (2002) Adult type vs. Childhood hepatocellular carcinoma – are they the same or different lesions? Biology, natural history, prognosis, and treatment. Med Pediatr Oncol 39(5):519–523

    Article  PubMed  CAS  Google Scholar 

  • Czauderna P, Mackinlay G et al (2002) Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group. J Clin Oncol 20(12):2798–2804

    Article  PubMed  CAS  Google Scholar 

  • Darbari A, Sabin KM et al (2003) Epidemiology of primary hepatic malignancies in U.S. children. Hepatology 38(3):560–566

    Article  PubMed  Google Scholar 

  • DeBaun MR, Tucker MA (1998) Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry. J Pediatr 132(3 Pt 1):398–400

    PubMed  CAS  Google Scholar 

  • Exelby PR, Filler RM et al (1975) Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey – 1974. J Pediatr Surg 10(3):329–337

    Article  PubMed  CAS  Google Scholar 

  • Haas JE, Feusner JH et al (2001) Small cell undifferentiated histology in hepatoblastoma may be unfavorable. Cancer 92(12):3130–3134

    Article  PubMed  CAS  Google Scholar 

  • Habrand JL, Nehme D et al (1992) 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):525–531

    Article  PubMed  CAS  Google Scholar 

  • Hirschman BA, Pollock BH et al (2005) The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds. J Pediatr 147(2):263–266

    Article  PubMed  CAS  Google Scholar 

  • Jassam N, Jones CM et al (2006) The hook effect: a need for constant vigilance. Ann Clin Biochem 43(Pt 4):314–317

    Article  PubMed  CAS  Google Scholar 

  • Katzenstein HM, Krailo MD et al (2002) Hepatocellular carcinoma in children and adolescents: results from the Pediatric Oncology Group and the Children’s Cancer Group intergroup study. J Clin Oncol 20(12):2789–2797

    Article  PubMed  Google Scholar 

  • Katzenstein HM, Kletzel M et al (2003a) Metastatic malignant rhabdoid tumor of the liver treated with tandem high-dose therapy and autologous peripheral blood stem cell rescue. Med Pediatr Oncol 40(3):199–201

    Article  PubMed  Google Scholar 

  • Katzenstein HM, Krailo MD et al (2003b) Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer 97(8):2006–2012

    Article  PubMed  Google Scholar 

  • Lin CY, Kuo CS et al (2010) Elevated serum vitamin B(12) levels in association with tumor markers as the prognostic factors predictive for poor survival in patients with hepatocellular carcinoma. Nutr Cancer 62(2):190–197

    Article  PubMed  CAS  Google Scholar 

  • Llovet JM, Ricci S, et al (2008) SHARP Investigators Study Group. Sorafenib in advanced hepatocellular carcinoma. N Engl J Med. Jul 24;359(4):378–90

    Article  CAS  Google Scholar 

  • Malogolowkin MH, Stanley P et al (2000) Feasibility and toxicity of chemoembolization for children with liver tumors. J Clin Oncol 18(6):1279–1284

    PubMed  CAS  Google Scholar 

  • Malogolowkin MH, Katzenstein HM et al (2008) Redefining the role of doxorubicin for the treatment of children with hepatoblastoma. J Clin Oncol 26(14):2379–2383

    Article  PubMed  CAS  Google Scholar 

  • Malogolowkin MH, Katzenstein HM, Krailo M, Rowland J, Haas J, Meyers RL, Finegold M (2011) Complete surgical resection is curative for children with hepatoblastoma with pure fetal histology. J Clin Oncol 29(24): 3301–3306

    Google Scholar 

  • Meyers RL, Katzenstein HM et al (2007) Surgical resection of pulmonary metastatic lesions in children with hepatoblastoma. J Pediatr Surg 42(12):2050–2056

    Article  PubMed  Google Scholar 

  • Ortega JA, Douglass EC et al (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):2665–2675

    PubMed  CAS  Google Scholar 

  • Otte JB, Pritchard J et al (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):74–83

    Article  PubMed  CAS  Google Scholar 

  • Perilongo G, Shafford E et al (2004) Risk-adapted treatment for childhood hepatoblastoma. Final report of the second study of the International Society of Paediatric Oncology–SIOPEL 2. International Society of Paediatric Oncology-SIOPEL 2. Eur J Cancer. Feb;40(3):411–21

    Article  Google Scholar 

  • Perilongo G, Maibach R et al (2009) Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma. N Engl J Med 361(17):1662–1670

    Article  PubMed  CAS  Google Scholar 

  • Pritchard J, Brown J et al (2000) 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):3819–3828

    PubMed  CAS  Google Scholar 

  • Prokurat A, Kluge P et al (2002) Transitional liver cell tumors (TLCT) in older children and adolescents: a novel group of aggressive hepatic tumors expressing beta-catenin. Med Pediatr Oncol 39(5):510–518

    Article  PubMed  Google Scholar 

  • Qayed M, Powell C et al (2010) Irinotecan as maintenance therapy in high-risk hepatoblastoma. Pediatr Blood Cancer 54(5):761–763

    PubMed  Google Scholar 

  • Ries LAG SM, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR (eds) (1999) Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. National Cancer Institute, Bethesda, SEER Program NIH Publication No. 99–4649

    Google Scholar 

  • Ross JA, Gurney JG (1998) Hepatoblastoma incidence in the United States from 1973 to 1992. Med Pediatr Oncol 30(3):141–142

    Article  PubMed  CAS  Google Scholar 

  • Rowland JM (2002) Hepatoblastoma: assessment of criteria for histologic classification. Med Pediatr Oncol 39(5):478–483

    Article  PubMed  Google Scholar 

  • Spector LG, Feusner JH et al (2004) Hepatoblastoma and low birth weight. Pediatr Blood Cancer 43(6):706

    Article  PubMed  Google Scholar 

  • Trobaugh-Lotrario AD, Tomlinson GE et al (2009) Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors. Pediatr Blood Cancer 52(3):328–334

    Article  PubMed  Google Scholar 

  • Van Tornout JM, Buckley JD et al (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):1190–1197

    PubMed  Google Scholar 

  • von Schweinitz D, Byrd DJ et al (1997) Efficiency and toxicity of ifosfamide, cisplatin and doxorubicin in the treatment of childhood hepatoblastoma. Study Committee of the Cooperative Paediatric Liver Tumour Study HB89 of the German Society for Paediatric Oncology and Haematology. Eur J Cancer 33(8):1243–1249

    Article  Google Scholar 

  • Wu JT, Book L et al (1981) Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr Res 15(1):50–52

    Article  PubMed  CAS  Google Scholar 

  • Zsíros J, Maibach R et al (2010) Successful treatment of ­childhood high-risk hepatoblastoma with dose-intensive multiagent chemotherapy and surgery: final results of the SIOPEL-3HR study J Clin Oncol. 2010 May 20;28(15):2584–90. Epub Apr 20

    Article  Google Scholar 

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Katzenstein, H.M., Casanova, M. (2012). Liver Tumors. In: Schneider, D., Brecht, I., Olson, T., Ferrari, A. (eds) Rare Tumors In Children and Adolescents. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-04197-6_36

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  • DOI: https://doi.org/10.1007/978-3-642-04197-6_36

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