Abstract
As documented in some of the chapters, in the last 3 decades, remarkable success has been achieved in the cure of hepatoblastoma (HB); in the late seventies the 5-year overall survival of children affected by this rare neoplasm was 30%; now it has more than doubled (Perilongo et al. 2009). Thus, at the beginning of this century, it can be stated that multidisciplinary approaches including conventional systemic chemotherapy has allowed the cure of more than two-third of the children diagnosed with hepatoblastoma. Remarkable refinements in the diagnostic capabilities, mainly related to the introduction of magnetic resonance images in daily clinical practice, in the surgical approach, in a more extensive and rational, use of orthotopic liver transplantation, in the supportive measures, in the use of systemic chemotherapy, and in tailoring treatment according to more and more individualized risk profiles are the facts that must be accounted to explain these gratifying results. However, it is a common assumption that to make further progress, that is, to cure those children affected by tumors, which are not amenable to any kind of radical surgery (including orthotopic liver transplantation), which are metastatic, do not respond to chemotherapy, recur, or present with low alpha-fetoprotein, different avenues must be envisaged.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Armengol C, Cairo S, Fabre M et al (2009) Wnt signaling and hepatocarcinogenesis: the hepatoblastoma model. Int J Biochem Cell Biol [Epub ahead of print 10.1016/j.biocel.2009.07.012
Cairo S, Armengol C, De Reyniès A et al (2008) Hepatic stem-like phenotype and interplay of Wnt/beta-catenin and Myc signaling in aggressive childhood liver cancer. Cancer Cell 14(6):471–484
Enguita-Germán M, Schiapparelli P, Rey JA et al (2010) CD133+ cells from medulloblastoma and PNET cell lines are more resistant to cyclopamine inhibition of the sonic hedgehog signaling pathway than CD133- cells. Tumour Biol 2010 May 18 [Epub ahead of print]
Lee EY, Ji H, Ouyang Z et al (2010) Hedgehog pathway-regulated gene networks in cerebellum development and tumorigenesis. Proc Natl Acad Sci U S A 107(21):9736–9741, Epub 2010 May 11
Lo Muzio L (2008) Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Orphanet J Rare Dis 3:32, Review
Pazzaglia S (2006) Ptc1 heterozygous knockout mice as a model of multi-organ tumorigenesis. Cancer Lett 234(2):124–134, Epub 2005 May 31. Review
Perilongo G, Maibach R, Shafford E et al (2009) Cisplatin versus cisplatin plus doxorubicin for standard risk hepatoblastoma. N Engl J Med 361(17):1662–1670
Si-Tayeb K, Lemaigre FP, Duncan SA (2010) Organogenesis and development of the liver. Dev Cell 18:175–189
Wandzioch E, Zaret KS (2009) Dynamic signaling network for the specification of embryonic pancreas and liver progenitors. Science 324:1707–1710
Wong SY, Reiter JF (2008) The primary cilium at the crossroads of mammalian hedgehog signaling. Curr Top Dev Biol 85:225–260
Zimmermann A (2005) The emerging family fo hepatoblastoma from ontogenesis to oncogenesis. Eur J Cancer 41(11):1503–1514, Review
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2011 Springer Berlin Heidelberg
About this chapter
Cite this chapter
Perilongo, G., Zimmermann, A. (2011). Future Prospective. In: Zimmermann, A., Perilongo, G. (eds) Pediatric Liver Tumors. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-14504-9_18
Download citation
DOI: https://doi.org/10.1007/978-3-642-14504-9_18
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-14503-2
Online ISBN: 978-3-642-14504-9
eBook Packages: MedicineMedicine (R0)