Hepatoblastoma pathophysiology: Difference between revisions
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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]]'', ''CDC20B''. On gross pathology, hepatoblastoma is characterized by well circumscribed large masses, usually single, with heterogeneous cut surface.<ref name=pathology>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 NC ratio, eosinophilic cytoplasm (mesenchymal component), 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), ''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== | ||
===Genetics=== | ===Genetics=== | ||
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==Pathogenesis== | ==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 favour the embryonic differentiation of the hepatoblastoma in certain conditions, this explaining the more frequent localization in the right hepatic lobe.<ref name="MadabhaviPatel2014">{{cite journal|last1=Madabhavi|first1=Irappa|last2=Patel|first2=Apurva|last3=Choudhary|first3=Mukesh|last4=Aagre|first4=Suhas|last5=Revannasiddaiah|first5=Swaroop|last6=Modi|first6=Gaurang|last7=Anand|first7=Asha|last8=Panchal|first8=Harsha|last9=Parikh|first9=Sonia|last10=Raut|first10=Shreeniwas|title=Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity|journal=Case Reports in Pediatrics|volume=2014|year=2014|pages=1–5|issn=2090-6803|doi=10.1155/2014/104543}}</ref> | 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 favour the embryonic differentiation of the hepatoblastoma in certain conditions, this explaining the more frequent localization in the right hepatic lobe.<ref name="MadabhaviPatel2014">{{cite journal|last1=Madabhavi|first1=Irappa|last2=Patel|first2=Apurva|last3=Choudhary|first3=Mukesh|last4=Aagre|first4=Suhas|last5=Revannasiddaiah|first5=Swaroop|last6=Modi|first6=Gaurang|last7=Anand|first7=Asha|last8=Panchal|first8=Harsha|last9=Parikh|first9=Sonia|last10=Raut|first10=Shreeniwas|title=Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity|journal=Case Reports in Pediatrics|volume=2014|year=2014|pages=1–5|issn=2090-6803|doi=10.1155/2014/104543}}</ref> | ||
==Gross Pathology== | ==Gross Pathology== | ||
On gross pathology, well circumscribed large masses, usually single, with heterogeneous cut surface are findings of hepatoblastoma.<ref name=pathology>Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref> | On gross pathology, well circumscribed large masses, usually single, with heterogeneous cut surface are findings of hepatoblastoma.<ref name=pathology>Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015</ref> | ||
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Image:Hepatoblastoma microscopy1.jpg|<sub>Micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.<ref>Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015</ref></sub> | Image:Hepatoblastoma microscopy1.jpg|<sub>Micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.<ref>Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015</ref></sub> | ||
</Gallery> | </Gallery> | ||
==Immunohistochemistry== | ==Immunohistochemistry== | ||
Hepatoblastoma is demonstrated by positivity to:<ref name=microscopy>IHC of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 4, 2015</ref> | Hepatoblastoma is demonstrated by positivity to:<ref name=microscopy>IHC of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 4, 2015</ref> | ||
*Alpha fetoprotein | *Alpha fetoprotein | ||
Revision as of 22:12, 4 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, CDC20B. On gross pathology, hepatoblastoma is characterized by well circumscribed large masses, usually single, with heterogeneous cut surface.[1] On microscopic histopathological analysis, hepatoblastoma is characterized by: small round cell tumor, fetal hepatocytes ~ 1:3 NC ratio, eosinophilic cytoplasm (mesenchymal component), immature fibrous tissue osteoid or cartilage.[2] Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, Hepatocyte specific antigen (especially in fetal component), Beta-catenin (cytoplasmic and nuclear)[2]
Pathophysiology
Genetics
Genes involved in the pathogenesis of hepatoblastoma include:[3]
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 favour the embryonic differentiation of the hepatoblastoma in certain conditions, this explaining the more frequent localization in the right hepatic lobe.[4]
Gross Pathology
On gross pathology, well circumscribed large masses, usually single, with heterogeneous cut surface are findings of hepatoblastoma.[1]
Microscopic Pathology
On microscopic histopathological analysis, hepatoblastoma is characterized by:[2]
- Small round cell tumor
- Fetal hepatocytes ~ 1:3 NC ratio, eosinophilic cytoplasm
- +/- Mesenchymal component
- Immature fibrous tissue, osteoid or cartilage
-
![Micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[5]](/images/0/09/Hepatoblastoma_microscopy1.jpg)
Micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[5]
![Micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.[5]](/index.php/File:Hepatoblastoma_microscopy1.jpg)
Immunohistochemistry
Hepatoblastoma is demonstrated by positivity to:[2]
- Alpha fetoprotein
- Hepatocyte specific antigen (especially in fetal component)
- Beta-catenin (cytoplasmic and nuclear)
References
- ↑ 1.0 1.1 Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
- ↑ 2.0 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
- ↑ 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.
- ↑ 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.
- ↑ Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015
