Hepatoblastoma

Definition

Definition of Hepatoblastoma
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. 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 Alpha-fetoprotein (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.

Symptoms

Symptoms of Hepatoblastoma
Symptoms at diagnosis can include:

1. abdominal lump or mass often located in the upper right side or middle of the abdomen
2. loss of appetite, nausea, abnormal weight loss, diarrhea
3. changes in the skin color: jaundice evidenced by yellow tone to the skin and whites of the eyes
4. general itching
5. fever, irritability, listlessness
6. abdominal pain

Causes

Causes of Hepatoblastoma
Although the exact cause of liver cancer is unknown, there are a number of genetic conditions that are associated with an increased risk for developing hepatoblastoma, including Beckwith-Wiedemann syndrome, hemihypertrophy, and familial adenomatous polyposis. Other genetic conditions associated with liver cancer include several inborn errors of metabolism such as tyrosinemia, glycogen storage disease type I, galactosemia, and alpha1-antitrypsin deficiency.

Children who are exposed to hepatitis B infection at an early age, or those who have biliary atresia, are also at increased risk for developing hepatocellular carcinoma. Some hepatoblastomas have genetic alterations in tumor suppressor genes, which would explain the uncontrolled cell growth.

Diagnosis

Diagnosis of Hepatoblastoma
In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:

1. Biopsy: a sample of tissue is removed from the tumor and examined under a microscope.
2. Complete Blood Count (CBC): a measurement of size, number, and maturity of different blood cells in a specific volume of blood.
3. Additional Blood Tests: may include blood chemistries, evaluation of liver and kidney functions, and genetic studies.

Treatment

Treatment of Hepatoblastoma
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. 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, adjuvant chemotherapy prior to resection, and liver transplantation 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.

Prognosis

Prognosis of Hepatoblastoma
Prognosis greatly depends on:

1. the extent of the disease.
2. the size and location of the tumor.
3. presence or absence of metastasis.
4. the tumor's response to therapy.
5. the age and overall health of your child.
6. your child's tolerance of specific medications, procedures, or therapies.
7. new developments in treatment.

Prevention

Prevention of Hepatoblastoma
Consult with your doctor.