Prof Gita Naidu educates us on hepatoblastoma, a rare childhood cancer of the liver.
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What is hepatoblastoma?
Hepatoblastoma is a cancer of the liver in children under three years of age and affects 1-2 million children globally.
- A painful lump in the middle or upper right side of the abdomen.
- Nausea and vomiting
- Loss of appetite
- Yellow-coloured skin or eyes
- Unexplained weight loss
What causes hepatoblastoma?
- Premature birth
- Low birth weight
- Early exposure to hepatitis B
- Biliary atresia
- Beckwith-Wiedemann syndrome
- Familial adenomatous polyposis
- Aicardi syndrome
- Glycogen storage disease
- Simpson-Golabi-Behmel syndrome
- Edward’s syndrome
How is hepatoblastoma diagnosed?
- Alpha-fetoprotein tumour marker test (sample of blood is taken).
- Imaging studies:
- Vascular ultrasound: blood vessels entering and leaving the liver
- Liver and doppler ultrasounds
Pre-treatment extent of disease (PRETEXT): the two lobes are divided into four sections. The groups are based on the number of sections with tumours.
- Upfront surgery to remove the tumour, followed by chemotherapy.
- Options for newly diagnosed hepatoblastoma that can’t be removed by surgery or is not removed at the time of diagnosis may include the following:
- Combination chemotherapy to shrink the tumour, followed by surgery to remove the tumour.
- Combination chemotherapy, followed by a liver transplant.
- Chemoembolisation of the hepatic artery to shrink the tumour, followed by surgery to remove the tumour.
- If the tumour in the liver can’t be removed by surgery but there are no signs of cancer in other parts of the body, the treatment may be a liver transplant.
- For newly diagnosed hepatoblastoma that has spread to other parts of the body at diagnosis:
- Combination chemotherapy to shrink the liver tumours and metastases.
- After chemotherapy, imaging tests are done to check whether the tumours can be removed by surgery. Treatment options may include the following:
- Surgery: If the tumour and metastases can be removed, followed by chemotherapy.
- If the metastases can’t be removed or a liver transplant isn’t possible, chemotherapy, chemoembolisation of the hepatic artery, or radiation therapy may be given.
Can hepatoblastoma be cured?
About 90% of children with early-stage disease are alive five years after treatment and about 75 to 85% of children with late-stage disease are alive five years after treatment.
Suggested questions for parents to ask
- What is the stage?
- What are treatment options?
- If surgery is an option, what surgery is recommended, and why?
- What are surgery side effects?
- Are there other treatments?
- Can these treatments cure my child?
- What is my child’s prognosis?
- What are the long-term complications of the disease and treatment?
MEET THE EXPERT – Professor Gita Naidu
Professor Gita Naidu MBChB, FC (Paediatrics), MMed (Paediatrics), PhD is the Head of Paediatric Oncology, Chris Hani Baragwanath Academic Hospital, Academic Head: Paediatric Oncology, University of the Witwatersrand and the Chair of South African Children’s Cancer Study Group.
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