Hepatoblastoma pathophysiology
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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]
- The exact pathogenesis of hepatoblastoma is not fully understood. [4]
- Loss of function mutations in APC leads to intracellular accumulation of the protooncogene beta-catenin, which leads to germline mutation of Wnt signal transduction and pathway.
- Hepatoblastomas originate from primitive hepatic stem cells.
- 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.[5]
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
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![Very high magnification micrograph of a hepatoblastoma. H&E stain.[7]](/images/0/09/Hepatoblastoma_microscopy1.jpg)
Very high magnification micrograph of a hepatoblastoma. H&E stain.[7]
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![High magnification micrograph of a hepatoblastoma. H&E stain.[8]](/images/a/ac/Hepatoblastoma_high_magnification.jpg)
High magnification micrograph of a hepatoblastoma. H&E stain.[8]
-
![High magnification micrograph of a hepatoblastoma. H&E stain.[9]](/images/3/34/Hepatoblastoma_high_magnification_2.jpg)
High magnification micrograph of a hepatoblastoma. H&E stain.[9]
![Very high magnification micrograph of a hepatoblastoma. H&E stain.[7]](/index.php/File:Hepatoblastoma_microscopy1.jpg)
![High magnification micrograph of a hepatoblastoma. H&E stain.[8]](/index.php/File:Hepatoblastoma_high_magnification.jpg)
![High magnification micrograph of a hepatoblastoma. H&E stain.[9]](/index.php/File:Hepatoblastoma_high_magnification_2.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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Hepatoblastoma. Librepathology (2015). http://librepathology.org/wiki/index.php/File:Hepatoblastoma_-_2_-_very_high_mag.jpg Accessed on November 3, 2015
- ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_high_mag.jpg Accessed on November 7, 2015
- ↑ Hepatoblastoma. Librepathology (2015). https://commons.wikimedia.org/wiki/File:Hepatoblastoma_-_2_-_high_mag.jpg Accessed on November 7, 2015
