Hepatoblastoma pathophysiology: Difference between revisions

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{{CMG}}; {{AE}} {{NM}}
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==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 NC 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===
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 favor 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>
*The exact [[pathogenesis]] of hepatoblastoma is not fully understood. <ref name="pmid19619488">{{cite journal |vauthors=MacDonald BT, Tamai K, He X |title=Wnt/beta-catenin signaling: components, mechanisms, and diseases |journal=Dev. Cell |volume=17 |issue=1 |pages=9–26 |date=July 2009 |pmid=19619488 |pmc=2861485 |doi=10.1016/j.devcel.2009.06.016 |url=}}</ref>
*Loss of function [[mutations]] in [[APC]] leads to [[intracellular]] accumulation of the [[protooncogene]] [[beta-catenin]], which leads to [[germline]] [[mutation]] of [[Wnt signalling pathway|Wnt signal]] [[transduction]] and pathway.
*Hepatoblastomas originate from primitive [[hepatic]] [[stem cells]].
*[[Beta-catenin|B-catenin]] [[mutations]] have been shown to be common in the majority of [[sporadic]] hepatoblastomas.
*Studies revealed that [[tumor]] occurs more often in families affected by [[familial adenomatous polyposis]]([[FAP]]), which is caused by inactivation of the [[adenomatous polyposis coli]] ([[APC]]), a [[tumor-suppressor gene]] that [[down-regulate]] the amount of [[beta-catenin]].
*[[Immunohistochemical]] markers such as expression of CK19, [[beta-catenin]] and EpCAM were correlated with [[tumor]] behaviour, response to [[chemotherapy]] and survival.<ref name="pmid29755772">{{cite journal |vauthors=Kiruthiga KG, Ramakrishna B, Saha S, Sen S |title=Histological and immunohistochemical study of hepatoblastoma: correlation with tumour behaviour and survival |journal=J Gastrointest Oncol |volume=9 |issue=2 |pages=326–337 |date=April 2018 |pmid=29755772 |pmc=5934143 |doi=10.21037/jgo.2018.01.08 |url=}}</ref>
==Genetics==
==Genetics==
Genes involved in the pathogenesis of hepatoblastoma include:<ref name="JiaDong2014">{{cite journal|last1=Jia|first1=Deshui|last2=Dong|first2=Rui|last3=Jing|first3=Ying|last4=Xu|first4=Dan|last5=Wang|first5=Qifeng|last6=Chen|first6=Lei|last7=Li|first7=Qigen|last8=Huang|first8=Yuping|last9=Zhang|first9=Yuannv|last10=Zhang|first10=Zhenfeng|last11=Liu|first11=Li|last12=Zheng|first12=Shan|last13=Xia|first13=Qiang|last14=Wang|first14=Hongyang|last15=Dong|first15=Kuiran|last16=He|first16=Xianghuo|title=Exome sequencing of hepatoblastoma reveals novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex|journal=Hepatology|volume=60|issue=5|year=2014|pages=1686–1696|issn=02709139|doi=10.1002/hep.27243}}</ref>
Genes involved in the pathogenesis of hepatoblastoma include:<ref name="JiaDong2014">{{cite journal|last1=Jia|first1=Deshui|last2=Dong|first2=Rui|last3=Jing|first3=Ying|last4=Xu|first4=Dan|last5=Wang|first5=Qifeng|last6=Chen|first6=Lei|last7=Li|first7=Qigen|last8=Huang|first8=Yuping|last9=Zhang|first9=Yuannv|last10=Zhang|first10=Zhenfeng|last11=Liu|first11=Li|last12=Zheng|first12=Shan|last13=Xia|first13=Qiang|last14=Wang|first14=Hongyang|last15=Dong|first15=Kuiran|last16=He|first16=Xianghuo|title=Exome sequencing of hepatoblastoma reveals novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex|journal=Hepatology|volume=60|issue=5|year=2014|pages=1686–1696|issn=02709139|doi=10.1002/hep.27243}}</ref>
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* ''CDC20B''
* ''CDC20B''
==Gross Pathology==
==Gross Pathology==
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 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>
==Microscopic Pathology==
==Microscopic Pathology==
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
===Gallery===
===Gallery===
<Gallery>
<gallery>
Image:
Image:
Hepatoblastoma microscopy1.jpg|<sub>Very high magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.<ref name=a>Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015</ref></sub>
Hepatoblastoma microscopy1.jpg|<sub>Very high magnification micrograph of a hepatoblastoma. H&E stain.<ref name=a>Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015</ref></sub>
Image:
Image:
Hepatoblastoma high magnification.jpg|<sub>High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.<ref name=b>Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_high_mag.jpg Accessed on November 7, 2015</ref></sub>
Hepatoblastoma high magnification.jpg|<sub>High magnification micrograph of a hepatoblastoma. H&E stain.<ref name=b>Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_high_mag.jpg Accessed on November 7, 2015</ref></sub>
Image:
Image:
Hepatoblastoma_high_magnification_2.jpg|<sub>High magnification micrograph of a hepatoblastoma, a type of liver cancer found in infants and young children. H&E stain.<ref name=c>Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_2_-_high_mag.jpg  Accessed on November 7, 2015</ref></sub>
Hepatoblastoma_high_magnification_2.jpg|<sub>High magnification micrograph of a hepatoblastoma. H&E stain.<ref name=c>Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_2_-_high_mag.jpg  Accessed on November 7, 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]]
*[[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}}
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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:[6]

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. H&E stain.[7]

    Very high magnification micrograph of a hepatoblastoma. H&E stain.[7]

  • High magnification micrograph of a hepatoblastoma. H&E stain.[8]

    High magnification micrograph of a hepatoblastoma. H&E stain.[8]

  • High magnification micrograph of a hepatoblastoma. H&E stain.[9]

    High magnification micrograph of a hepatoblastoma. H&E stain.[9]

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. ↑ MacDonald BT, Tamai K, He X (July 2009). "Wnt/beta-catenin signaling: components, mechanisms, and diseases". Dev. Cell. 17 (1): 9–26. doi:10.1016/j.devcel.2009.06.016. PMC 2861485. PMID 19619488.
  5. ↑ Kiruthiga KG, Ramakrishna B, Saha S, Sen S (April 2018). "Histological and immunohistochemical study of hepatoblastoma: correlation with tumour behaviour and survival". J Gastrointest Oncol. 9 (2): 326–337. doi:10.21037/jgo.2018.01.08. PMC 5934143. PMID 29755772.
  6. ↑ 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.
  7. ↑ Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015
  8. ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_high_mag.jpg Accessed on November 7, 2015
  9. ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_2_-_high_mag.jpg Accessed on November 7, 2015


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