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Hepatoblastoma

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Hepatoblastoma
Micrographof a hepatoblastoma.H&E stain.
SpecialtyOncologyEdit this on Wikidata

Hepatoblastomais a malignantliver canceroccurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, orbile ductcells. 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]

Signs and symptoms

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

Pathophysiology

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Micrographshowing a hepatoblastoma (right of image) and normal liver (left of image).H&E stain.
Liver tumor types by relative incidence in adults in the United States, with hepatoblastoma at right[4]

Hepatoblastomas originate from immature liver precursor cells, are typically unifocal, affect the right lobe of thelivermore often than the left lobe, and canmetastasize.They are categorized into two types: "Epithelial Type" and "Mixed Epithelial / Mesenchymal Type."[citation needed]

Individuals withfamilial adenomatous polyposis(FAP), a syndrome of early-onsetcolonicpolypsandadenocarcinoma,frequently develop hepatoblastomas.[5][6]Also,beta-cateninmutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.[7][8]

Recently, other components of theWnt signaling pathwayhave also demonstrated a likely role in constitutive activation of this pathway in the causation of hepatoblastoma.[8][9]Accumulating evidence suggests that hepatoblastoma is derived from a pluripotentstem cell.[10]

Syndromes with an increased incidence of hepatoblastoma includeBeckwith–Wiedemann syndrome,trisomy 18, trisomy 21,Acardi syndrome,Li–Fraumeni syndrome,Goldenhar syndrome,von Gierke disease, and familial adenomatous polyposis.[11]

Diagnosis

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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 second 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 in adults. 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.[12]

Treatment

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Surgicalremoval of the tumor,neoadjuvantchemotherapyprior to tumor removal, andliver transplantationhave been used to treat these cancers.[13][14]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%.[15][16]The presence of metastases is the strongest predictor of a poor prognosis.[17]

References

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  1. ^"Archived copy".Archived fromthe originalon 2019-05-31.Retrieved2010-09-15.{{cite web}}:CS1 maint: archived copy as title (link)[full citation needed]
  2. ^De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, et al. (March 2008). "Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience".European Journal of Cancer.44(4): 545–550.doi:10.1016/j.ejca.2007.11.022.PMID18166449.
  3. ^Willert, Jennifer."Pediatric Hepatoblastoma Clinical Presentation: History, Physical, Causes".emedicine.medscape.com.
  4. ^Table 37.2in:Sternberg, Stephen (2012).Sternberg's diagnostic surgical pathology.Place of publication not identified: LWW.ISBN978-1-4511-5289-0.OCLC953861627.
  5. ^Hirschman BA, Pollock BH, Tomlinson GE (August 2005). "The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds".The Journal of Pediatrics.147(2): 263–266.doi:10.1016/j.jpeds.2005.04.019.PMID16126064.
  6. ^Sanders RP, Furman WL (November 2006). "Familial adenomatous polyposis in two brothers with hepatoblastoma: implications for diagnosis and screening".Pediatric Blood & Cancer.47(6): 851–854.doi:10.1002/pbc.20556.PMID16106429.S2CID34824663.
  7. ^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 Research.60(11): 2864–2868.PMID10850429.
  8. ^abTan X, Apte U, Micsenyi A, Kotsagrelos E, Luo JH, Ranganathan S, et al. (July 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.PMC1821080.PMID16012954.
  9. ^Koch A, Waha A, Hartmann W, Hrychyk A, Schüller U, Waha A, et al. (June 2005)."Elevated expression of Wnt antagonists is a common event in hepatoblastomas".Clinical Cancer Research.11(12): 4295–4304.doi:10.1158/1078-0432.CCR-04-1162.PMID15958610.
  10. ^Ruck P, Xiao JC (November 2002). "Stem-like cells in hepatoblastoma".Medical and Pediatric Oncology.39(5): 504–507.doi:10.1002/mpo.10175.PMID12228907.
  11. ^Zimmerman A, Saxena R. Hepatoblastoma. In: WHO Classification of Tumours of the Digestive System, 4th, Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds), IARC, Lyon 2010. p.229.
  12. ^Sarto I, Klausberger T, Ehya N, Mayer B, Fuchs K, Sieghart W (August 2002)."A novel site on gamma 3 subunits important for assembly of GABA(A) receptors".The Journal of Biological Chemistry.277(34): 30656–30664.doi:10.1074/jbc.M203597200.PMID12065588.
  13. ^Ang JP, Heath JA, Donath S, Khurana S, Auldist A (February 2007). "Treatment outcomes for hepatoblastoma: an institution's experience over two decades".Pediatric Surgery International.23(2): 103–109.doi:10.1007/s00383-006-1834-1.PMID17119981.S2CID11332782.
  14. ^Otte JB, Pritchard J, Aronson DC, Brown J, Czauderna P, Maibach R, 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".Pediatric Blood & Cancer.42(1): 74–83.doi:10.1002/pbc.10376.PMID14752798.S2CID20741138.
  15. ^Pediatric HepatoblastomaateMedicine
  16. ^Otte JB, de Ville de Goyet J, Reding R (October 2005). "Liver transplantation for hepatoblastoma: indications and contraindications in the modern era".Pediatric Transplantation.9(5): 557–565.doi:10.1111/j.1399-3046.2005.00354.x.PMID16176410.S2CID74339.
  17. ^Czauderna P, Mackinlay G, Perilongo G, Brown J, Shafford E, Aronson D, et al. (June 2002)."Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group".Journal of Clinical Oncology.20(12): 2798–2804.doi:10.1200/JCO.2002.06.102.PMID12065556.Archived fromthe originalon 2013-04-15.
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  • humpath#2775(Pathology images)
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