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{{CMG}}; {{AE}} {{NM}}
{{CMG}}; {{AE}} {{NM}}
==Overview==
==Overview==
Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include ''[[Beta-catenin|CTNNB1]]'', ''CAPRIN2'', ''[[SPOP]]'', ''[[OR5I1]]'', and ''CDC20B''. On gross pathology, hepatoblastoma is characterized by a solitary, large, well circumscribed mass with heterogeneous cut surface.<ref name=differrential>Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref> On microscopic histopathological analysis, hepatoblastoma is characterized by small round cell tumor, fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio ratio, eosinophilic cytoplasm (mesenchymal component), and immature fibrous tissue osteoid or cartilage.<ref name=microscopy>Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015</ref> Hepatoblastoma is demonstrated by positivity to [[alpha-fetoprotein]], hepatocyte specific antigen (especially in fetal component), and ''[[beta-catenin]]'' (cytoplasmic and nuclear).<ref name=microscopy>IHC of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 4, 2015</ref>
Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include ''[[Beta-catenin|CTNNB1]]'', ''CAPRIN2'', ''[[SPOP]]'', ''[[OR5I1]]'', and ''CDC20B''. On gross pathology, hepatoblastoma is characterized by a solitary, large, well circumscribed mass with heterogeneous cut surface.<ref name=differrential>Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref> On microscopic histopathological analysis, hepatoblastoma is characterized by small round cell tumor, fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio, eosinophilic cytoplasm (mesenchymal component), and immature fibrous tissue osteoid or cartilage.<ref name=microscopy>Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015</ref> Hepatoblastoma is demonstrated by positivity to [[alpha-fetoprotein]], hepatocyte specific antigen (especially in fetal component), and beta-catenin (cytoplasmic and nuclear).<ref name=microscopy>IHC of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 4, 2015</ref>
==Pathophysiology==
==Pathophysiology==
==Pathogenesis==
==Pathogenesis==
Line 19: Line 19:
On microscopic histopathological analysis, hepatoblastoma is characterized by:<ref name=microscopy>Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015</ref>
On microscopic histopathological analysis, hepatoblastoma is characterized by:<ref name=microscopy>Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015</ref>
* Small round cell tumor
* Small round cell tumor
* Fetal hepatocytes ~ 1:3 NC ratio, eosinophilic cytoplasm
* Fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio, eosinophilic cytoplasm
* +/- Mesenchymal component
* +/- Mesenchymal component
** Immature fibrous tissue, osteoid, or cartilage
** Immature fibrous tissue, osteoid, or cartilage
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*[[Alpha fetoprotein]]
*[[Alpha fetoprotein]]
*[[Hepatocyte specific antigen]] (especially in fetal component)
*[[Hepatocyte specific antigen]] (especially in fetal component)
*''[[Beta-catenin]]'' (cytoplasmic and nuclear)
*Beta-catenin (cytoplasmic and nuclear)
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 19:15, 16 November 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

Overview

Development of hepatoblastoma is the result of multiple genetic mutations. Genes involved in the pathogenesis of hepatoblastoma include CTNNB1, CAPRIN2, SPOP, OR5I1, and CDC20B. On gross pathology, hepatoblastoma is characterized by a solitary, large, well circumscribed mass with heterogeneous cut surface.[1] On microscopic histopathological analysis, hepatoblastoma is characterized by small round cell tumor, fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio, eosinophilic cytoplasm (mesenchymal component), and immature fibrous tissue osteoid or cartilage.[2] Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, hepatocyte specific antigen (especially in fetal component), and beta-catenin (cytoplasmic and nuclear).[2]

Pathophysiology

Pathogenesis

Hepatoblastoma usually develops in the right hepatic lobe. The left hepatic lobe receives oxygenated blood from the umbilical vein, while the right lobe receives oxygenated blood from the portal vein, with lower oxygen saturation. The lower oxygen saturation could favor the embryonic differentiation of the hepatoblastoma in certain conditions, this explaining the more frequent localization in the right hepatic lobe.[3]

Genetics

Genes involved in the pathogenesis of hepatoblastoma include:[4]

Gross Pathology

On gross pathology, hepatoblastoma is characterized by a solitary, large, well circumscribed mass with heterogeneous cut surface.[1]

Microscopic Pathology

On microscopic histopathological analysis, hepatoblastoma is characterized by:[2]

  • Small round cell tumor
  • Fetal hepatocytes ~ 1:3 nuclear-cytoplasmic ratio, eosinophilic cytoplasm
  • +/- Mesenchymal component
    • Immature fibrous tissue, osteoid, or cartilage

Gallery

  • Very high magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[5]
    Very high magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[5]
  • High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[6]
    High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[6]
  • High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[7]
    High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[7]

Immunohistochemistry

Hepatoblastoma is demonstrated by positivity to:[2]

References

  1. ↑ 1.0 1.1 Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
  2. ↑ 2.0 2.1 2.2 2.3 Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015
  3. ↑ Madabhavi, Irappa; Patel, Apurva; Choudhary, Mukesh; Aagre, Suhas; Revannasiddaiah, Swaroop; Modi, Gaurang; Anand, Asha; Panchal, Harsha; Parikh, Sonia; Raut, Shreeniwas (2014). "Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity". Case Reports in Pediatrics. 2014: 1–5. doi:10.1155/2014/104543. ISSN 2090-6803.
  4. ↑ Jia, Deshui; Dong, Rui; Jing, Ying; Xu, Dan; Wang, Qifeng; Chen, Lei; Li, Qigen; Huang, Yuping; Zhang, Yuannv; Zhang, Zhenfeng; Liu, Li; Zheng, Shan; Xia, Qiang; Wang, Hongyang; Dong, Kuiran; He, Xianghuo (2014). "Exome sequencing of hepatoblastoma reveals novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex". Hepatology. 60 (5): 1686–1696. doi:10.1002/hep.27243. ISSN 0270-9139.
  5. ↑ Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015
  6. ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_high_mag.jpg Accessed on November 7, 2015
  7. ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_2_-_high_mag.jpg Accessed on November 7, 2015


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