Hepatoblastoma overview
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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
Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal or mature liver cells or bile ducts. They usually present with an abdominal mass. Alpha-fetoprotein (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.[1]
Historical Perspective
Hepatoblastoma was first described in 1958 by I. Bartok.[2]
Classification
Hepatoblastoma may be classified into two subtypes based on histology, major category and minor category.[3]
Pathophysiology
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.[4] 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.[5] Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, Hepatocyte specific antigen (especially in fetal component), Beta-catenin (cytoplasmic and nuclear)[5]
Causes
There are no established causes for hepatoblastoma. The etiology of hepatoblastoma has been elusive.[2]
Epidemiology and Demographics
Hepatoblastoma is a common tumor that tends to affect children aged less than five years.[6] The annual incidence of hepatoblastoma in the United States appears to have doubled from 0.8 (1975-1983) to 1.6 (2002-2009) per 1 million children aged 19 years and younger.[7]
Risk factors
To view a comprehensive list of risk factors that increase the risk of hepatoblastoma, click here.[7]
Screening
Differentiating Hepatoblastoma from other diseases
Hepatoblastoma must be differentiated from other diseases such as hepatic mesenchymal hamartoma, hepatocellular carcinoma, hepatic metastases, infantile hemangioendothelioma, rhabdomyosarcoma of biliary tract.[8]
Natural History, Complications and Prognosis
Diagnosis
Staging
History and Symptoms
Physical examination
Laboratory Findings
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ De Ioris M, Brugieres L, Zimmermann A, Keeling J, Brock P, Maibach R, Pritchard J, Shafford L, Zsiros J, Czaudzerna P, Perilongo G (2007). "Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience". Eur J Cancer. 44: 545. doi:10.1016/j.ejca.2007.11.022. PMID 18166449.
- ↑ 2.0 2.1 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.
- ↑ Radiopaedia 2015 Hepatoblastoma>"Radiopedia 2015 Hepatoblastoma [Dr Frank Gaillard]".
- ↑ Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
- ↑ 5.0 5.1 Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015
- ↑ Allan, Bassan J.; Parikh, Punam P.; Diaz, Sofia; Perez, Eduardo A.; Neville, Holly L.; Sola, Juan E. (2013). "Predictors of survival and incidence of hepatoblastoma in the paediatric population". HPB. 15 (10): 741–746. doi:10.1111/hpb.12112. ISSN 1365-182X.
- ↑ 7.0 7.1 Incidence. National Cancer Institute 2015. http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#link/_570_toc. Accessed on October 28, 2015
- ↑ Differential diagnosis of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015