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Liver cancers in children

Hepatoblastoma and hepatocellular carcinoma

Marcus Malogolowkin is an MD at Children’s Hospital in Los Angeles.

He wrote the following article and I have included it here for people who are trying to understand Baby Ella Frantz’s disease. Baby Ella has hepatoblastoma. As far as I know decisions regarding surgery for Ella have not yet been determined. Ella’s disease was discovered when she arrived at her pediatricians for a regular well baby checkup at the tender age of  2 months old. Since December 2009 she has endured 4 sessions of chemotherapy:

The liver is an organ found in the upper right side of the abdomen, protected by the rib cage.  When cells that make up the liver grow without the usual controls that prevent growth, liver cancer may develop. The two most common types of liver cancers in children are:

  • Hepatoblastoma occurs most frequently in infants or very young children between the ages of 2 months and 3 years. This is the most common kind of cancer of the liver in children.
  • Hepatocellular carcinoma (HCC) occurs most frequently in children between the ages of 10 and 16 years.

Together hepatoblastoma and HCC account for about 1-2% of cancers in children.

What causes a liver tumor?

The liver is a large, very important organ. The normal functions of the liver in the body are:

  • To help store nutrients from food
  • To break down and remove harmful chemicals from the body
  • To build chemicals that the body needs to stay healthy

It is unclear exactly how hepatoblastoma develops, but it is believed to develop when mistakes (mutations) occur during the growth of liver cells. These cells grow without the usual regulation of normal liver cells.  This uncontrolled growth leads to the development of a liver tumor.

Hepatocellular carcinoma is increased in areas of the world that have a high incidence of hepatitis.  Infection with any one of several viruses that cause hepatitis is believed to be responsible.

What are the symptoms of liver cancer?

Liver cancer most often causes enlargement of the abdomen due to the rapidly enlarging tumor. This often happens without any other symptoms. When additional symptoms do occur they may include:

  • Loss of appetite
  • Weight loss
  • Vomiting
  • Stomach pain
  • Jaundice (rare)
  • Unequal growth of one part of the body compared to another (for example one leg larger than another)
  • Early signs of puberty

Blood tests that can be affected by liver cancer include:

  • The Complete Blood Count (CBC)
    • An increased number of platelets called thrombocytosis – The platelets are needed by the body for clotting; an excess of platelets can lead to bleeding problems.
    • A decreased number of red blood cells called anemia – Red blood cells carry oxygen throughout the body.
  • Alphafetoprotein (AFP) is a substance (protein) made by immature (early in development) liver cells.  It is often elevated in children with liver tumors.

What are the factors affecting prognosis?

Factors that affect treatment type and cure rate include:

  • Complete tumor resection (removal)
    Children whose tumors can be completely removed by surgery are most likely to be cured.through a surgical procedure are most likely to be cured. However, some tumors cannot be completely removed at the time of diagnosis. In these cases, it is often recommended that chemotherapy be given prior to surgery to shrink the tumor and improve the chances of complete removal.
  • Presence of “pure fetal histology”
    After a biopsy or surgical removal of the tumor, pathologist who has experience with hepatoblastoma examines the tumor tissue and determines whether the tumor cells resemble those present in a fetal liver  (pure fetal histology). These children have an excellent cure rate if their tumors have been completely resected.
  • Tumor stage
    Earlier stage tumors (I or II) are more easily cured. However, 70% of liver tumors are late stage (III or IV) by the time they are diagnosed.
  • Alpha-fetoprotein (AFP) production
    Most liver tumors produce a substance called AFP. In children who have an abnormally high AFP at diagnosis and who are initially treated with chemotherapy, a rapid decrease in AFP levels may be associated with a favorable cure rate.  Those who have a normal or slightly elevated AFP do not do as well and may require different treatment.

You can click here to read more about my tiny friend, Baby Ella Frantz, who has this disease.

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