Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal or mature liver cells or bile duct

Bile duct

A bile duct is any of a number of long tube-like structures that carry bile.Bile, required for the digestion of food, is excreted by the liver into passages that carry bile toward the hepatic duct, which joins with the cystic duct to form the common bile duct, which opens into the intestine.The...

s. Affecting 1 in 1.5 million. They are usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life http://hepatoblastoma.mditv.com/generalinfoAlpha-fetoprotein

Alpha-fetoprotein

Alpha-fetoprotein is a protein that in humans is encoded by the AFP gene....

 (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.

Pathophysiology

Hepatoblastomas originate from immature liver precursor cells, usually unifocal and affect the right lobe of the liver

Liver

The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...

 more often than the left lobe, can metastasize.

Patients with familial adenomatous polyposis

Familial adenomatous polyposis

Familial adenomatous polyposis is an inherited condition in which numerous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon cancer occurs when not treated....

 (FAP

FAP

FAP may refer to:Technology and industry* FORTRAN Assembly Program, the macro assembler for some IBM mainframe computers* Fair Access Policy, a term for a bandwidth cap, limiting Internet usage...

), a syndrome of early-onset colon

Colon (anatomy)

The colon is the last part of the digestive system in most vertebrates; it extracts water and salt from solid wastes before they are eliminated from the body, and is the site in which flora-aided fermentation of unabsorbed material occurs. Unlike the small intestine, the colon does not play a...

ic polyps and adenocarcinoma

Adenocarcinoma

Adenocarcinoma is a cancer of an epithelium that originates in glandular tissue. Epithelial tissue includes, but is not limited to, the surface layer of skin, glands and a variety of other tissue that lines the cavities and organs of the body. Epithelium can be derived embryologically from...

, frequently develop hepatoblastomas. Also beta-catenin

Beta-catenin

Beta-catenin is a protein that in humans is encoded by the CTNNB1 gene. In Drosophila, the homologous protein is called armadillo...

 mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.

Recently on other components of the Wnt signaling pathway

Wnt signaling pathway

The Wnt signaling pathway is a network of proteins best known for their roles in embryogenesis and cancer, but also involved in normal physiological processes in adult animals.-Discovery:...

 have also demonstrated a likely role for constitutive activation of this pathway in the etiology of hepatoblastoma.

Accumulating evidence suggests that hepatoblastoma is derived from a pluripotent stem cell

Stem cell

This article is about the cell type. For the medical therapy, see Stem Cell TreatmentsStem cells are biological cells found in all multicellular organisms, that can divide and differentiate into diverse specialized cell types and can self-renew to produce more stem cells...

.

One study suggests that low birth weight might be a risk factor for hepatoblastoma.

Treatment

The most common method of testing for hepatoblastoma is to a blood test checking the Alpha-fetoprotein. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children. When levels of AFP are too high the risk the individual has for liver cancer sky rockets. At birth, infants have relatively high levels of AFP, which fall to normal adult levels by the first year of life. The Normal levels for AFP in children has been reported as lower than 50 nanograms per milliliter (ng/ml) and 10 ng/ml. AFP Blood Test AFP greater than 500 (ng/ml) is a significant indicator of hepatoblastoma. AFP is also used as an indicator of the success of treatments. If treatments are successful in removing the cancer it can be expected that AFP levels will fall back to normal.

Surgical resection

Segmental resection

Segmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...

, adjuvant

Adjuvant

An adjuvant is a pharmacological or immunological agent that modifies the effect of other agents, such as a drug or vaccine, while having few if any direct effects when given by itself...

 chemotherapy

Chemotherapy

Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

 prior to resection, and liver transplantation

Liver transplantation

Liver transplantation or hepatic transplantation is the replacement of a diseased liver with a healthy liver allograft. The most commonly used technique is orthotopic transplantation, in which the native liver is removed and replaced by the donor organ in the same anatomic location as the original...

have been used to treat these neoplasms, primary transplantation provides high, long term, disease-free survival rate in the range of 80%, in cases of complete resection and adjuvant chemotherapy survival rates approach 100%. The presence of metastases is the most potent predictor of poor prognosis.

External links

• humpath #2775 (Pathology images)
• University of Minnesota hepatoblastoma epidemiology study
• What is Hepatoblastoma?