Hepatoblastoma: Description, Causes and Risk Factors:HepatoblastomaA malignant neoplasm occurring in young children, primarily in the liver, composed of tissue resembling embryonal or fetal hepatic epithelium, or mixed epithelial and mesenchymal tissues.Hepatoblastoma is a solid tumor cancer of the liver. This primary liver tumor which accounts for half of all liver tumors in children is rare with approximately 50 to 70 new cases diagnosed in the United States each year. It occurs slightly more often in boys than girls.Like most cancers, scientists do not know the cause of this disease. Some children however are at an increased risk of being diagnosed with hepatoblastoma due to other genetic conditions. These include: Beckwith-Wiedemann syndrome, familial adenomatous polyposis, and Aicardi syndrome.Stage:Stage I — Usually a tumor that can be completely removed with surgery.
  • Stage II — Usually a tumor that can mostly be removed by surgery; very small amounts of the cancer are left in the liver.
  • Stage III — Usually a tumor that cannot be completely removed, or else; cancer cells are also have been found in the lymph nodes.
  • Stage IV — Cancer that has spread to other parts of the body.
  • Recurrent — The disease has returned after it has been treated; i.e., It may come back in the liver or in another part of the body.
Hepatoblastoma is predominantly found in preschool aged children. Half of all patients are diagnosed in their first year of life, with the majority of patients being diagnosed by their third birthday.Primary epithelial liver tumor in children are relatively uncommon - they constitute about 1-1.5% of all pediatric neoplasm. The annual incidence of hepatoblastoma is about 1.5 cases per million children aged less than 15 years. No significant geographic variation in the overall incidence of hepatoblastoma exists throughout the world. The median age at presentation is about 24 months with no sex prevalence. Congenital cases have been reported.Symptoms:Abdominal lump or mass often located in the upper right side or middle of the abdomen.
  • Loss of appetite, nausea, abnormal weight loss, diarrhea.
  • Changes in the skin color: jaundice evidenced by yellow tone to the skin and whites of the eyes.
  • General itching.
  • Fever, irritability.
  • Abdominal pain.
Diagnosis:In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:Complete blood count (CBC) — A measurement of size, number, and maturity of different blood cells in a specific volume of your blood.
  • Additional blood tests — Blood chemistries, evaluation of liver and kidney functions, and genetic study.
  • Biopsy — A sample of tissue removed from the tumor and examined under a microscope; your surgeon may also look at the liver using an instrument called a peritoneoscope, a small tube with a light on the end.
  • Alpha-fetoprotein (AFP) test — Alpha-fetoprotein (AFP) levels in the blood can be used to diagnose and follow your child's response to treatment.
  • Serum tumor marker tests: Hepatoblastoma often produces a protein which can be detected in the blood. Levels of alpha-fetoprotein (AFP) can be used as a way to determine whether the tumor is responding to treatment (AFP levels decrease), whether there is no evidence of disease, or if there is an indication of relapse (increased levels of AFP).
Multiple imaging studies, including:X-ray — A diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs on film.
  • Computerized tomography scan (also called a CT or CAT scan) — A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
  • Magnetic resonance imaging (MRI) — A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
  • Ultrasound (also called sonography) — Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
  • Liver scans — Pictures or x-rays taken after a dye has been injected that is absorbed by liver tissue. These are used to detect tumors and liver abnormalities.
Treatment:Once hepatoblastoma has been diagnosed, the tumor will be staged based upon the extent of the tumor and whether it can be surgically removed. Approximately half of all hepatoblastoma tumors diagnosed are small and can be successfully treated with surgery alone (Stage I). The liver is one organ which is capable of regenerating itself, thus making surgery the best chance for a complete cure. In cases where the tumor is large and disease has spread to the lymph nodes (Stage III), or has spread to other parts of the body, primarily the lung (Stage IV), chemotherapy is used to shrink the tumor prior to surgical resection. Children whose tumors have relapsed and have not spread outside of the liver could be candidates for a Liver transplant.Hepatoblastoma responds well to chemotherapy agents when used to shrink tumors prior to surgery. Chemotherapy agents include cisplatin, fluorouracil, doxorubicin (Adriamycin® & RubexTM), and vincristine. Other possible agents used are ifosfamide, dactinomycin, and etoposide (ToposarTM).NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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