Rare Primary Care News

Disease Profile

Childhood hepatocellular carcinoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

US Estimated

Europe Estimated

Age of onset

Childhood

ageofonset-childhood.svg

ICD-10

C22.0

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Childhood Carcinoma of Liver Cell; Childhood Hepatoma; Childhood Liver Cell Carcinoma;

Categories

Digestive Diseases; Rare Cancers

Summary

Childhood hepatocellular carcinoma (childhood HCC) is a rare type of malignant (cancerous) tumor that forms in the cells and tissues of the liver.[1] Childhood HCC is usually found in older children and adolescents (10-14 years), but has been found in children younger than 5.[2] Symptoms may include a mass in the abdomen, a swollen and painful abdomen, weight loss, poor appetite, yellowing of the skin and eyes (jaundice), vomiting, fever, itchy skin, and a decreased number of red blood cells (anemia).[3][1] The cause of childhood HCC is not well understood; however, underlying disorders that cause liver dysfunction (ex. hepatitis B) and congenital or genetic disorders that affect the liver (ex. tyrosinemia type 1) may increase the risk for childhood HCC to occur.[1][2] Treatment options vary depending on a variety of factors including the stage of the cancer and may include surgery to remove the tumor and chemotherapy.[1]

Symptoms

The symptoms of childhood hepatocellular carcinoma (childhood HCC) are similar to the symptoms found in other liver cancers, commonly presenting with an abdominal mass and pain. Other common symptoms include abdominal swelling and discomfort, weight loss, anorexia, fever, vomiting, and yellowing of the skin and eyes (jaundice).[3][1][2]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain

[ more ]

 0002027
Elevated alpha-fetoprotein
0006254
Hepatic fibrosis
0001395
Hepatomegaly
Enlarged liver
0002240
30%-79% of people have these symptoms
Epigastric pain
0410019
Fatigue
Tired
Tiredness

[ more ]

 0012378
Hepatic necrosis
0002605
Portal vein thrombosis
Blood clot in portal vein
0030242
Vomiting
Throwing up
0002013
Percent of people who have these symptoms is not available through HPO
Hepatocellular carcinoma
0001402
Micronodular cirrhosis
0001413
Somatic mutation
0001428
Subacute progressive viral hepatitis
0006572

Cause

The cause of childhood hepatocellular carcinoma (childhood HCC) is not well understood. Unlike adult HCC, childhood HCC may be found in individuals with no underlying liver disease.[1]

Children living in regions of the world where the hepatitis B virus is common have been reported to have a much greater risk of developing this disease. Chronic infection by hepatitis C virus has also been linked to the development of childhood HCC.[4] Childhood HCC has also been reported to develop in the presence of liver disease, cirrhosis, and genetic disorders such as tyrosinemia type 1, glycogen storage disease type 1, and glycogen storage disease type IV.[2]

In addition, various other reported risk factors for developing childhood HCC include: male sex, family history of this carcinoma, and exposure to aflatoxin by food contamination.[4][3]

Treatment

The treatment for childhood hepatocellular carcinoma (childhood HCC) is dependent on a number of factors including the PRETEXT and POSTTEXT group. This is a way of describing the tumor before treatment (PRETEXT) and after treatment (POSTTEXT). The course of treatment is additionally dependent on whether or not a tumor can be initially removed with surgery and whether the cancer has spread (metastasized) to other parts of the body.[2][5] 

Treatment options for a tumor that can be removed by surgery at the time of diagnosis may include:[2][5]

  • Surgery alone to remove the tumor
  • Surgery and chemotherapy
  • Combination chemotherapy followed by surgery to remove the tumor

Treatment options for a tumor that cannot be removed by surgery at the time of diagnosis may include:[2][5][1]

Treatment for a childhood HCC that has spread to other parts of the body may include a combination of chemotherapy and surgery to remove as much of the tumor from the liver as possible as well as other places where the cancer has spread. If the childhood HCC is related to a hepatitis B viral infection, treatment may additionally include antiviral medications.[5]

Management Guidelines

  • The National Cancer Institute offers information for health professionals on the treatment of childhood liver cancer including Childhood hepatocellular carcinoma

    Organizations

    Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

    Organizations Supporting this Disease

      Organizations Providing General Support

        Learn more

        These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

        Where to Start

        • Children's Hospital Boston Web site has an information page on hepatocellular carcinoma, childhood. Click on the link above to view this information page.
        • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
        • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
        • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

          In-Depth Information

          • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
          • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
          • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
          • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
          • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
          • PubMed is a searchable database of medical literature and lists journal articles that discuss Childhood hepatocellular carcinoma. Click on the link to view a sample search on this topic.

            References

            1. Pr Piotr CZAUDERNA. Pediatric hepatocellular carcinoma. Orphanet. November 2014; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=33402.
            2. Deirdre Kelly, Khalid Sharif, Rachel M. Brown, Bruce Morland. Hepatocellular Carcinoma in Children. Clin Liver Dis. May 2015; 19(2):433-447. https://www.ncbi.nlm.nih.gov/pubmed/25921672.
            3. Paulette Mehta. Pediatric Hepatocellular Carcinoma. Medscape. November 5, 2015; https://emedicine.medscape.com/article/986988.
            4. Rosenthal P. Hepatocarcinoma in viral and metabolic liver disease.. Journal of Pediatric Gastroenterology and Nutrition. 2008; https://www.ncbi.nlm.nih.gov/pubmed/18367947. Accessed 7/14/2016.
            5. Childhood Liver Cancer Treatment (PDQ®)–Patient Version. National Cancer Institute. June 20, 2016; https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq#link/_247_toc.

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