Hepatoblastoma: Difference between revisions
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'''Hepatoblastoma''' is an uncommon malignant [[liver neoplasm]] occurring in infants and children and composed of tissue resembling fetal or mature liver cells or [[bile duct]]s. They are usually present with an abdominal mass. [[Alpha-fetoprotein]] (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.<ref name="pmid18166449">{{cite journal |
'''Hepatoblastoma''' is an uncommon malignant [[liver neoplasm]] occurring in infants and children and composed of tissue resembling fetal or mature liver cells or [[bile duct]]s. They are usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life[http://hepatoblastoma.mditv.com/generalinfo][[Alpha-fetoprotein]] (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.<ref name="pmid18166449">{{cite journal |
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| author = De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G |
| author = De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G |
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| title = Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience. |
| title = Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience. |
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==References== |
==References== |
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{{reflist}} |
{{reflist}} |
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*[http://hepatoblastoma.mditv.com/generalinfo] |
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== External links == |
== External links == |
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Revision as of 23:00, 15 September 2010
| Hepatoblastoma | |
|---|---|
| Specialty | Oncology  |
Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal or mature liver cells or bile ducts. They are usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life[1]Alpha-fetoprotein (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.[1]
Pathophysiology
Hepatoblastomas originate from immature liver precursor cells, usually unifocal and affect the right lobe of the liver more often than the left lobe, can metastasize.
Patients with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas [2][3]. Also beta-catenin mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients[4][5].
Recently on other components of the Wnt signaling pathway have also demonstrated a likely role for constitutive activation of this pathway in the etiology of hepatoblastoma[5][6].
Accumulating evidence suggests that hepatoblastoma is derived from a pluripotent stem cell[7].
Recently Studies suggested that low birth weight might be a risk factor for hepatoblastoma[8].
Treatment
The most common method of testing for hepatoblastoma is to a blood test checking the Alpha-fetoprotein. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children. When levels of AFP are too high the risk the individual has for liver cancer sky rockers. At birth, infants have relatively high levels of AFP, which fall to normal adult levels by the first year of life. The Normal levels for AFP in children has been reported as lower than 50 nanograms per milliliter (ng/ml) and 10 ng/ml. AFP Blood Test AFP greater than 500 (ng/ml) is a significant indicator of hepatoblastoma. AFP is also used as an indicator of the success of treatments. If treatments are successful in removing the cancer it can be expected that AFP levels will fall back to normal.[9]
Surgical resection, adjuvant chemotherapy prior to resection, and liver transplantation have been used to treat these neoplasms[10][11], primary transplantation provides high, long term, disease-free survival rate in the range of 80%, in cases of complete resection and adjuvant chemotherapy survival rates approach 100% [12] [13]. The presence of metastases is the most potent predictor of poor prognosis[14].
References
- ^ De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G (2007). "Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience". Eur J Cancer. 44 (4): 545. doi:10.1016/j.ejca.2007.11.022. PMID 18166449.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Hirschman BA, Pollock BH, Tomlinson GE (2005). "The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds". J. Pediatr. 147 (2): 263–6. doi:10.1016/j.jpeds.2005.04.019. PMID 16126064.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Sanders RP, Furman WL (2006). "Familial adenomatous polyposis in two brothers with hepatoblastoma: implications for diagnosis and screening". Pediatr Blood Cancer. 47 (6): 851–4. doi:10.1002/pbc.20556. PMID 16106429.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Anna CH, Sills RC, Foley JF, Stockton PS, Ton TV, Devereux TR (2000). "Beta-catenin mutations and protein accumulation in all hepatoblastomas examined from B6C3F1 mice treated with anthraquinone or oxazepam". Cancer Res. 60 (11): 2864–8. PMID 10850429.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ a b Tan X, Apte U, Micsenyi A; et al. (2005). "Epidermal growth factor receptor: a novel target of the Wnt/beta-catenin pathway in liver". Gastroenterology. 129 (1): 285–302. doi:10.1053/j.gastro.2005.04.013. PMC 1821080. PMID 16012954.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Koch A, Waha A, Hartmann W; et al. (2005). "Elevated expression of Wnt antagonists is a common event in hepatoblastomas". Clin. Cancer Res. 11 (12): 4295–304. doi:10.1158/1078-0432.CCR-04-1162. PMID 15958610.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Ruck P, Xiao JC (2002). "Stem-like cells in hepatoblastoma". Med. Pediatr. Oncol. 39 (5): 504–7. doi:10.1002/mpo.10175. PMID 12228907.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Tanimura M, Matsui I, Abe J; et al. (1998). "Increased risk of hepatoblastoma among immature children with a lower birth weight". Cancer Res. 58 (14): 3032–5. PMID 9679968.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Tim, P; et al. "Hepatoblastoma".
{{cite journal}}: Cite journal requires|journal=(help); Explicit use of et al. in:|author=(help) - ^ Ang JP, Heath JA, Donath S, Khurana S, Auldist A (2007). "Treatment outcomes for hepatoblastoma: an institution's experience over two decades". Pediatr. Surg. Int. 23 (2): 103–9. doi:10.1007/s00383-006-1834-1. PMID 17119981.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Otte JB, Pritchard J, Aronson DC; 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. doi:10.1002/pbc.10376. PMID 14752798.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ http://emedicine.medscape.com/article/986802-overview
- ^ 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): 557–65. doi:10.1111/j.1399-3046.2005.00354.x. PMID 16176410.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Czauderna P, Mackinlay G, Perilongo 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): 2798–804. doi:10.1200/JCO.2002.06.102. PMID 12065556.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link)
External links
- humpath #2775 (Pathology images)
- University of Minnesota hepatoblastoma epidemiology study