Hepatoblastoma

About Hepatoblastoma

Hepatoblastoma is the most common malignant liver tumor in less than 3 years of age and the median age of diagnosis is 18 months of life. There are other liver masses and one needs to differentiate between benign and malignant tumors, as treatment varies accordingly.


 

Why does it occur?

Though pediatric cancers do not have clear evidence of exogenous factors causing the tumor to occur, Hepatoblastoma has been associated with low birth weight babies (<1500gms) babies and parental smoking. The syndromes associated with it are trisomy 18, Beckwith Weidman syndrome and Familial Adenomatosis Polyposis

 

Clinical features

Abdominal distension/mass incidentally detected by a primary physician.
Advanced/metastatic disease anorexia, pain, weight loss, and breathlessness

 

How do we diagnose?

Routine blood tests
Tumor markers – Alpha-fetoprotein (AFP), for age less than one year follow age for AFP level.
Imaging – Both contrasts enhanced computerized tomography (CECT) and magnetic resonance imaging (MRI) help in defining the tumor extension and relation to blood vessels and metastasis. CT scan of the thorax is required to rule out metastasis to the lungs.
Tissue diagnosis- biopsy is required when tumor markers and radiological features are not suggestive of Hepatoblastoma especially in children below 6 months of age to more than 3 years.

PRETEXT staging
The staging of Hepatoblastoma is based on the initial imaging done at presentation and the extent of disease is defined in numbers. This is called as PRETEXT (Pre-treatment extent of disease) staging and also has other additional criteria which radiologist assesses on imaging. This helps to identify high-risk features on imaging, assess surgical challenges if present, define prognosis and plan effective treatment.

PRETEXT stage Definition
I One section is involved and three adjoining sections are free
II One or two sections are involved, but two adjoining sections are free
III Two or three sections are involved, and no two adjoining sections are free
IV All four sections are involved

Risk stratification
High-risk Standard risk
Serum alpha-fetoprotein <100 μg/l

PRETEXT IV
Additional PRETEXT criteria:
E1, E1a, E2, E2a
H1
M1 (any site)
N1, N2
P2, P2a
V3, V3a All other patients

 

Treatment

The treatment of Hepatoblastoma includes chemotherapy and surgery. With the help of radiology, the staging (PRETEXT) is done and the intensity of chemotherapy depends on the risk stratification. The chemotherapy includes single-agent cisplatin for standard risk and combination of cisplatin, carboplatin, and doxorubicin for high-risk patients. The chemotherapy drugs have side effects which need to be monitored, so the additional investigation is required as 2-D ECHO( cardiac) based on the drug used. The surgery is performed preferably after 4- 6 cycles after assessment of the disease. The serum AFP level is monitored during the complete treatment period.

Liver transplant – The liver transplant means that clinical situation where a liver transplant is an option, and Follow up After completion of treatment, the child is followed up with serum AFP levels and other investigations based on chemotherapy drugs given. The elevation in AFP levels mandates radiological imaging to identify recurrence if any. The follow-up visits initially are more frequent and then later the interval is increased.

 

Outcomes

The outcomes of standard risk and high-risk disease range between 85–95% and 56-82% in amongst major groups worldwide.

 

Useful links

  • https://link.springer.com/article/10.1007/s00383-015-3763-3
  • https://www.sciencedirect.com/science/article/pii/S0959804911010367
  • https://www.ncbi.nlm.nih.gov/pubmed/25904441
  • https://www.ncbi.nlm.nih.gov/pubmed/20406943
  • https://www.ncbi.nlm.nih.gov/pubmed/22244829
  • https://www.ncbi.nlm.nih.gov/pubmed/25336801

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