What is Hepatoblastoma?

Hepatoblastoma is a rare tumor of liver. It is however the most common cancerous liver tumor in early childhood. Usually affects children younger than 3 years more so younger than 18 months. The frequency is more in low birth weight babies.

How does Hepatoblastoma present? What are symptoms of Hepatoblastoma?

  • Abdominal distension especially upper abdomen
  • Yellowish discolouration of eyes (jaundice)
  • Loss of appetite
  • Weight loss, Vomiting
  • Other symptoms: Back pain, fever, early sexual development( rare cases)

How is Hepatoblastoma diagnosed?

Hepatoblastoma is suspected based on history and mass in upper right abdomen.

Initial diagnosis is made based on blood tests and radiological investigations like:

  • Alpha feto protein levels (blood test) : AFP is synthesized by the liver and serves as tumor marker for hepatoblastoma. However levels need to be interpreted with age based nomograms. AFP can be elevated in several other conditions affecting liver. Hence values should be evaluated in that context. It also serves as a marker for monitoring treatment.
  • Sonography abdomen : Helps to know about the location, extent of the mass.
  • CT Scan of abdomen / MRI abdomen : Helps to know about the location (part of liver involved), extent (size), spread of the tumor if any, its effect on surrounding structure. It also helps to stage the tumor and know the operability.
  • Biopsy : It’s a procedure where a tiny piece of liver mass is collected from suspected areas either sonography or CT guided technique after sedating the child. This tissue (piece of liver) is processed and examined under microscope to diagnose the tumor, its type (histology), etc.
  • Bone scan : It is a radiological technique to scan the bone to detect any abnormal signals in the bones. This helps us to detect the spread of the tumor to bones, indicating that the tumor is advanced.

What is staging of the tumor?

Staging of the tumor is done preoperatively by CT/ MRI abdomen to know the extent of the disease (spread within liver and outside like lung, bones), to decide about treatment plan. There two staging systems like PRETEXT staging and COG (Children’s Oncology Group).

What is the treatment of Hepatoblastoma?

Treatment involves surgery and chemotherapy.

  • Chemotherapy is used to shrink the size of the tumor and treat the tumor that has spread outside liver. Chemotherapy involves giving medicines (Cisplatin, Vincristine, Doxorubicin) that destroy the cancer cells. It is given at particular interval through an injection/ drip. The child needs to follow certain precautions about hygiene and food habits to avoid infections as are directed by the pediatric oncologist
  • Surgery also called tumor resection involves removing the tumor from child’s liver such that there is enough normal liver tissue left to maintain function. This depends upon the location of tumor within the liver, whether it is pressing upon vital structures, causing any obstruction, etc.

 

About 30 % children may have limited disease and surgery can be considered at diagnosis and later followed by chemotherapy. However sometimes in advanced tumors few cycles of chemotherapy may be given prior to surgery to shrink the tumor size (Neoadjuvant chemotherapy).

The treatment approach is individualized and planned after discussing with co specialities and the parents.

 

  • Liver Transplant

Transplant is the only treatment option for unresectable/ inoperable/ advanced tumors.

 

Survival rates in Hepatoblastoma

5 year survival rate is more than 80 % if the tumor is only in the liver and can be completely removed with surgery after chemotherapy. Survival rates for children whose tumor involves entire liver or have spread outside the liver ranges from 20 to 70 % at 5 years.

References 

 

1.

Primary malignant liver tumors in children.

 

Agarwala S.

 

Indian J Pediatr. 2012 Jun;79(6):793-800. doi: 10.1007/s12098-012-0704-1. Epub 2012

 

 

 

 

2.

Outcomes of hepatoblastoma in the Indian context.

 

Arora RS.

 

Indian Pediatr. 2012 Apr;49(4):307-9.

 

 

 

 

3.

Survival and long-term outcomes in children with hepatoblastoma treated with continuous infusion of cisplatin and doxorubicin.

 

Sivaprakasam P, Gupta AA, Greenberg ML, Capra M, Nathan PC.

 

J Pediatr Hematol Oncol. 2011 Aug.

 

 

 

 

4.

Hepatoblastoma: experience from a single center.

 

Singh T, Satheesh CT, Appaji L, Aruna Kumari BS, Padma M, Kumar MV, Mukherjee G.

 

Indian J Cancer. 2010 Jul-Sep.

 

 

 

 

5.

Hepatoblastoma: a single institutional experience of 18 cases.

 

Shukla PJ, Barreto SG, Qureshi SS, Hawaldar R, Shrikhande SV, Ramadwar MR, Banavali S.

 

Pediatr Surg Int. 2008 Jul;24(7):799-802.

 

 

 

 

6.

Neoadjuvant chemotherapy before surgery of hepatoblastoma.

 

Udupa KV, Navadgi SM, Mullerpatan P, Chhabra D, Shah RC, Jagannath P.

 

Indian J Pediatr. 2006 Aug;73(8):735-7.

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