Treatment for Hepatoblastoma varies on a case by case basis with many factors influencing the protocol including stage, location, health and age. The primary forms of treatment are surgery and chemotherapy, with radiotherapy considered for more serious cases where options are limited.
The standard protocol in Europe, where the prognosis for ‘standard risk’ cases is the highest in the world at 75%, is to shrink the primary tumour with chemotherapy and then remove it via surgery (resection).
For ‘high risk’ cases, where the prognosis is around 30%, the same initial protocol will be followed and the initial surgery will either be a resection, or in cases where the entire liver is compromised, a liver transplant. If the cancer has spread (metasteses), most likely to the lungs, then after surgery further chemotherapy is required to attempt to clear the tumours. ‘High risk’ cases with lung metasteses are the most complex to cure and have the lowest prognosis because lung metasteses can be extremely difficult to remove, even with surgery, if there are many widespread nodules.
Chemotherapy drugs are highly toxic to the body and work by killing off fast dividing cells by preventing them from dividing. Unfortunately this also affects healthy cells that divide rapidly such as hair, stomach lining, bone marrow etc. so the side effects can be severe leading to hair loss, suppressed imune system, blood transfusions, diarrhea etc.
Of particular importance during treatment is monitoring the blood counts; heamaglobin (HB), platelets and neutrophils:
- Heamaglobin are classic red blood cells and responsible for transferring oxygen around the body. These are normally above 10 but if below 7 you will be lethargic and pale and require a blood transfusion.
- Platelets allow the blood to clot – normal levels go into the 1000’s and most chemotherapy or surgery has a minimum platelet level of 100. If platelets drop below 20 a transfusion will be needed however, these are not recommended as the platelets will only survive for a day or so. It’s crucial that the body is producing its own platelets.
- Neutrophils are the white blood cells that fight infection. Normal counts are well over 5. If the count drops below 1 you have neutropenia and become suceptible to infections. If the count drops to 0.1 or below you are severely neutropenic and must be admitted to hospital in isolation until the count comes up. Most chemotherapy and surgery sets a minimum level of 1 for neutrophils. Thankfully, there is a drug called GCSF which stimulates the bone marrow to generate neutrophils very quickly to ensure levels remain high.
Cisplatin, Doxorubicin and Carboplatin are high toxicity and highly effective 1st line treatment. Duration of usage is limited to months as they can cause long term damage to the heart, kidneys and hearing.
Irinotecan and Vincristine are lower toxicity but still effective 2nd line treatment. Duration of usage can be several years.
The liver is an incredible organ, with the ability to regenerate itself within days. This is why the prognosis for ‘standard risk’ cases is so high. In a liver resection the primary tumour is removed plus an amount of surrounding healthy tissue to ensure nothing is left behind. It’s possible to remove 75% of the liver without serious complications and it will regenerate to full strength within weeks. In the UK, Kings College Hospital is the liver specialist and most resections are carried out there.
The lungs are notoriously difficult to operate on. There are five lobes; two on the left and three on the right. If there are only a few tumours (nodules) in the lungs a thoracotamy and wedge resection can be carried out, which involves opening the back or chest and removing the tissue in the offending areas. Alternatively, if a particular lobe(s) is heavily affected a lobectamy can be performed to remove entire lobes. If the tumours are widespread in all lobes the only option is to perform a thoracotamy and work through the entirity of all lobes to remove any tumours, though there is a risk some microscopic tumours could be missed.