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 a malignant liver neoplasm that occurs infants and children. It is composed of tissue resembling fetal or mature liver cells or bile ducts. Hepatoblastoma was first described in 1958 by I. Bartok.[1] It may be classified into two categories based on histology: major category and minor category.[2] Development of hepatoblastoma is the result of multiple genetic mutations. On gross pathology, it is characterized by a well-circumscribed, large, single mass with a heterogeneous cut surface.[3] There are no established causes for hepatoblastoma.[1] Hepatoblastoma is a common tumor that tends to affect children aged less than five years.[4] The annual incidence of hepatoblastoma in the United States is thought to be increasing.[5] Hepatoblastoma must be differentiated from other diseases such as hepatic mesenchymal hamartoma, hepatocellular carcinoma, hepatic metastases, infantile hemangioendothelioma, and rhabdomyosarcoma of biliary tract.[3] If left untreated, patients with hepatoblastoma may progress to develop failure to thrive, weight loss, rapidly enlarging abdominal mass, spontaneous rupture, hemorrhage, and death.[1] The staging of hepatoblastoma is based on the Intergroup staging system.[6] Symptoms of hepatoblastoma include painless lump in the abdomen, abdominal pain, and weight loss.[1][3] Common physical examination findings of hepatoblastoma include hepatomegaly, asymptomatic palpable abdominal mass, hemihypertrophy, jaundice, pyrexia, and anemia.[1][3] Laboratory tests for liver function are usually normal. Abdominal CT scan may be helpful in the diagnosis of hepatoblastoma. Findings on CT scan suggestive of hepatoblastoma include well defined heterogeneous mass, frequent areas of necrosis and hemorrhage, and chunkycdense calcifications.[3] Other diagnostic studies for hepatoblastoma include biopsy. The predominant therapy for hepatoblastoma is surgical resection. Neoadjuvant and adjuvant chemotherapy may be required.[1] Surgery is the mainstay of treatment for hepatoblastoma.[1] Screening is recommended among patients with Beckwith-Wiedemann syndrome or patients with isolated hemihyperplasia. Screening measures include routine abdominal ultrasound and measurement of serum alpha-fetoprotein concentration.[5]
Historical Perspective
Hepatoblastoma was first described in 1958 by I. Bartok.[1]
Classification
Hepatoblastoma may be classified into two groups based on histology, major category and minor category.[2]
Pathophysiology
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.[7] 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.[8] Hepatoblastoma is demonstrated by positivity to alpha-fetoprotein, hepatocyte specific antigen (especially in fetal component), and beta-catenin (cytoplasmic and nuclear).[8]
Causes
There are no established causes for hepatoblastoma.[1]
Epidemiology and Demographics
Hepatoblastoma is a common tumor that tends to affect children aged less than five years.[4] Males are more commonly affected with hepatoblastoma than females.[3] The annual incidence of hepatoblastoma in the United States appears to have doubled from 8 (1975-1983) to 16 (2002-2009) per 100,000 children aged 19 years and younger.[5]
Risk factors
To view a comprehensive list of risk factors that increase the risk of hepatoblastoma, click here.[5]
Screening
According to the American Association for the Study of Liver Diseases and United States Preventive Services Task Force, there is insufficient evidence to recommend routine screening for hepatoblastoma in the general population.[9][10] Effective measures for the secondary prevention of hepatoblastoma include use of abdominal ultrasound and alpha-fetoprotein levels in patients with Beckwith-Wiedemann syndrome or isolated hemihyperplasia.[5]
Differentiating Hepatoblastoma from other diseases
Hepatoblastoma must be differentiated from other diseases such as hepatic mesenchymal hamartoma, hepatocellular carcinoma, hepatic metastases, infantile hemangioendothelioma, and rhabdomyosarcoma of biliary tract.[3]
Natural History, Complications and Prognosis
If left untreated, patients with hepatoblastoma may progress to develop failure to thrive, weight loss, rapidly enlarging abdominal mass, spontaneous rupture, hemorrhage and death.[1] Common complications of hepatoblastoma include paraneoplastic features, erythrocytosis, thrombocytosis, hypocalcemia, isosexual precocious puberty, hypoglycemia.[1] The 5-year survival rate of children with hepatoblastoma is approximately 70%.[5]
Diagnosis
Staging
The staging of hepatoblastoma is based on the Intergroup staging system.[6]
History and Symptoms
Symptoms of hepatoblastoma include painless lump in the abdomen, abdominal pain, weight loss, loss of appetite, early puberty in boys, jaundice, nausea, vomiting, back pain, failure to thrive, and rapidly enlarging abdominal mass.[1][3]
Physical examination
Common physical examination findings of hepatoblastoma include hepatomegaly, abdominal distension, asymptomatic palpable abdominal mass, hemihypertrophy, jaundice, pyrexia, and anemia.[1][3]
Laboratory Findings
Laboratory tests for liver function are usually normal. An elevated concentration of alpha-fetoprotein is present in patients with hepatoblastoma.[1]
Xray
Findings on abdominal xray are nonspecific for hepatoblastoma and include right upper quadrant abdominal mass and/or calcifications in 10% of cases.[3]
CT
Abdominal CT scan may be helpful in the diagnosis of hepatoblastoma. Findings on CT scan suggestive of hepatoblastoma include well defined heterogeneous mass, frequent areas of necrosis and hemorrhage, and chunky dense calcifications.[3]
MRI
Abdominal MRI is helpful in the diagnosis of hepatoblastoma. On MRI, hepatoblastoma is characterized by hypointensity on T1-weighted imaging and hyperintensity compared to liver with areas of necrosis and hemorrhage on T2-weighted imaging.[3]
Ultrasound
Ultrasound may be helpful in the diagnosis of hepatoblastoma. Findings on ultrasound suggestive of hepatoblastoma include echogenic soft tissue mass, well defined lesion, and areas of shadowing due to intralesional calcifications.[3]
Other Diagnostic Studies
Other diagnostic studies for hepatoblastoma include biopsy. Histopathological findings on biopsy can be found here.[1]
Treatment
Medical Therapy
The predominant therapy for hepatoblastoma is surgical resection. Neoadjuvant and adjuvant chemotherapy may be required.[1]
Surgery
Surgery is the mainstay of treatment for hepatoblastoma.[1]
Primary Prevention
There are no primary preventive measures available for hepatoblastoma.
Secondary Prevention
Effective measures for the secondary prevention of hepatoblastoma include use of abdominal ultrasound and alpha-fetoprotein levels in patients with Beckwith-Wiedemann syndrome or isolated hemihyperplasia.[5]
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 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.
- ↑ 2.0 2.1 Classification of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia (2015). http://radiopaedia.org/articles/hepatoblastoma-histological-classification. Accessed on November 3, 2015
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
- ↑ 4.0 4.1 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.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Incidence. National Cancer Institute 2015. http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#link/_570_toc. Accessed on October 28, 2015
- ↑ 6.0 6.1 Intergroup staging system of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma-staging-2. Accessed on November 3, 2015
- ↑ Pathology of hepatoblastoma. Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/hepatoblastoma. Accessed on November 3, 2015
- ↑ 8.0 8.1 Microscopic features of hepatoblastoma. Librepathology (2015). Accessed on http://librepathology.org/wiki/index.php/Liver_neoplasms#Hepatoblastoma. November 3, 2015
- ↑ Hepatoblastoma. USPSTF. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=hepatoblastoma Accessed on November 3, 2015
- ↑ Hepatoblastoma. AASLD. https://www.aasld.org/search/node/hepatoblastoma%20screening Accessed on November 3, 2015