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Hepatoblastoma


Source: http://en.wikipedia.org/wiki/Hepatoblastoma
Updated: 2017-03-26T05:05Z
Hepatoblastoma
Hepatoblastoma - 2 - very high mag.jpg
Micrograph of a hepatoblastoma. H&E stain.
Classification and external resources
Specialty{{#statements:P1995}}
ICD-10C22.0
ICD-OM8970/3
DiseasesDB5799
eMedicineped/982 radio/331
Patient UKHepatoblastoma
MeSHD018197
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Hepatoblastoma is an uncommon malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They 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) levels are commonly elevated, but when AFP is not elevated at diagnosis the prognosis is poor.[2]

Symptoms

Patients are usually asymptomatic[3] at diagnosis. As a result, disease is often advanced at diagnosis.

Pathophysiology

Micrograph showing a hepatoblastoma (right of image) and normal liver (left of image). H&E stain.

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. They are categorized into two types: "Epithelial Type" and "Mixed Epithelial / Mesenchymal Type."

Individuals with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas.[4][5] Also beta-catenin mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.[6][7]

Recently on other components of the Wnt signaling pathway have also demonstrated a likely role for constitutive activation of this pathway in the cause of hepatoblastoma.[7][8]Accumulating evidence suggests that hepatoblastoma is derived from a pluripotent stem cell.[9]

Diagnosis

The most common method of testing for hepatoblastoma is a blood test checking the alpha-fetoprotein level. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children. At birth, infants have relatively high levels of AFP, which fall to normal adult levels by the first year of life. The normal level for AFP in children has been reported as lower than 50 nanograms per milliliter (ng/ml) and 10 ng/ml. An AFP level greater than 500 (ng/ml) is a significant indicator of hepatoblastoma. AFP is also used as an indicator of treatment success. If treatments are successful in removing the cancer, the AFP level is expected to return to normal.[10]

Treatment

Surgical removal of the tumor, adjuvant chemotherapy prior to tumor removal, and liver transplantation have been used to treat these cancers.[11][12] Primary liver transplantation provides high, long term, disease-free survival rate in the range of 80%, in cases of complete tumor removal and adjuvant chemotherapy survival rates approach 100%.[13][14] The presence of metastases is the strongest predictor of a poor prognosis.[15]

References

  1. ^ http://hepatoblastoma.mditv.com/generalinfo[full citation needed]
  2. ^ 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): 545–50. PMID 18166449. doi:10.1016/j.ejca.2007.11.022. 
  3. ^ Willert, Jennifer. "Pediatric Hepatoblastoma Clinical Presentation: History, Physical, Causes". emedicine.medscape.com. 
  4. ^ Hirschman BA, Pollock BH, Tomlinson GE (August 2005). "The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds". J. Pediatr. 147 (2): 263–6. PMID 16126064. doi:10.1016/j.jpeds.2005.04.019. 
  5. ^ Sanders RP, Furman WL (November 2006). "Familial adenomatous polyposis in two brothers with hepatoblastoma: implications for diagnosis and screening". Pediatr Blood Cancer. 47 (6): 851–4. PMID 16106429. doi:10.1002/pbc.20556. 
  6. ^ Anna CH, Sills RC, Foley JF, Stockton PS, Ton TV, Devereux TR (June 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. 
  7. ^ a b Tan, Xinping; Apte, Udayan; Micsenyi, Amanda; Kotsagrelos, Emorphia; Luo, Jian-Hua; Ranganathan, Sarangarajan; Monga, Dulabh K.; Bell, Aaron; Michalopoulos, George K.; Monga, Satdarshan P.S. (2005). "Epidermal Growth Factor Receptor: A Novel Target of the Wnt/β-Catenin Pathway in Liver". Gastroenterology. 129 (1): 285–302. PMC 1821080Freely accessible. PMID 16012954. doi:10.1053/j.gastro.2005.04.013. 
  8. ^ Koch A, Waha A, Hartmann W, et al. (June 2005). "Elevated expression of Wnt antagonists is a common event in hepatoblastomas". Clin. Cancer Res. 11 (12): 4295–304. PMID 15958610. doi:10.1158/1078-0432.CCR-04-1162. 
  9. ^ Ruck P, Xiao JC (November 2002). "Stem-like cells in hepatoblastoma". Med. Pediatr. Oncol. 39 (5): 504–7. PMID 12228907. doi:10.1002/mpo.10175. 
  10. ^ Sarto, I.; Klausberger, T.; Ehya, N.; Mayer, B.; Fuchs, K.; Sieghart, W. (2002). "A novel site on gamma 3 subunits important for assembly of GABA(A) receptors". The Journal of Biological Chemistry. 277 (34): 30656–64. PMID 12065588. doi:10.1074/jbc.M203597200. 
  11. ^ Ang JP, Heath JA, Donath S, Khurana S, Auldist A (February 2007). "Treatment outcomes for hepatoblastoma: an institution's experience over two decades". Pediatr. Surg. Int. 23 (2): 103–9. PMID 17119981. doi:10.1007/s00383-006-1834-1. 
  12. ^ Otte JB, Pritchard J, Aronson DC, et al. (January 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. PMID 14752798. doi:10.1002/pbc.10376. 
  13. ^ Pediatric Hepatoblastoma at eMedicine
  14. ^ Otte JB, de Ville de Goyet J, Reding R (October 2005). "Liver transplantation for hepatoblastoma: indications and contraindications in the modern era". Pediatr Transplant. 9 (5): 557–65. PMID 16176410. doi:10.1111/j.1399-3046.2005.00354.x. 
  15. ^ Czauderna P, Mackinlay G, Perilongo G, et al. (June 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. PMID 12065556. doi:10.1200/JCO.2002.06.102. 

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