Hepatoblastoma Surgery - Liver Cancer Surgery Child
Hepatoblastoma surgery removes the most common liver cancer of young children, after cisplatin chemotherapy has shrunk the tumor. Depending on PRETEXT stage, the surgeon removes part of the liver or, when the tumor cannot be resected, replaces it with a living donor transplant. At Biruni Hospital in Istanbul, our pediatric oncology, liver surgery and transplant teams plan each child's treatment together, protect hearing during chemotherapy and follow every child with AFP tests.
About This Department
Pediatric Surgery
Hepatoblastoma is the most common liver cancer in young children. Chemotherapy shrinks it, and complete surgical removal cures most children.
Hepatoblastoma surgery removes a liver cancer that starts in the immature liver cells of babies and young children, with a peak before the age of three. Surgery decides cure. Treatment combines cisplatin chemotherapy before and after the operation with complete removal of the tumor, either by taking out part of the liver or, when the tumor fills all four sections of the liver or surrounds its main veins, by liver transplantation. In a European trial of 255 children with standard risk tumors, cisplatin alone allowed complete removal in 95 percent, with three-year survival of 95 percent (Perilongo and colleagues, New England Journal of Medicine, 2009). A later trial found that adding sodium thiosulfate after each cisplatin dose cut hearing loss from 63 to 33 percent without lowering survival (Brock and colleagues, New England Journal of Medicine, 2018). This page explains signs, PRETEXT staging, risk groups, chemotherapy, hearing protection, liver resection, transplantation, recovery, follow-up, and how families from abroad arrange hepatoblastoma surgery at Biruni Hospital in Istanbul.
95
Percent three-year survival with cisplatin and surgery for standard risk hepatoblastoma
48
Percent fewer cases of hearing loss when sodium thiosulfate follows cisplatin
80
Percent long-term survival after primary transplant for tumors that cannot be resected
Free
Review of your child's scans and AFP results by our pediatric liver team
Hepatoblastoma is rare, one to two children in a million each year, and most are diagnosed before their third birthday. With modern treatment, the large majority are cured.
Children at higher risk
Factor
Why it matters
What we advise
Very low birth weight or prematurity
Higher rate of hepatoblastoma
Attention to any belly swelling
Beckwith-Wiedemann syndrome
Overgrowth condition with tumor risk
AFP and ultrasound every three months to age four
Familial adenomatous polyposis in the family
APC gene change
Genetic counseling
Not the adult liver cancer
Hepatoblastoma differs from the liver cancer of adults. It arises in a healthy liver, without cirrhosis or hepatitis, and responds well to chemotherapy. Most tumors make a protein called alpha-fetoprotein, AFP, which serves as a tumor marker in the blood.
1
The cure depends on surgery
Chemotherapy alone does not cure hepatoblastoma. Every cured child has had the whole tumor removed, by resection or transplant.
Signs and first tests
How hepatoblastoma shows itself
Sign
What parents notice
Note
Swollen belly
Firm lump under the right ribs
Most common first sign
Poor appetite or weight loss
Less feeding, tiredness
Larger tumors
Pale skin
Anemia
From bleeding into the tumor
Sudden pain and pallor
Rupture of the tumor
Emergency
Which tests come first?
Blood tests for AFP, liver function and blood counts, an ultrasound, then an MRI or CT of the abdomen and a CT of the chest. A needle biopsy confirms the diagnosis and the tumor subtype before chemotherapy starts. Infants under six months have high AFP normally, so we compare results against age-specific values.
First tests and their purpose
Test
Purpose
Time
AFP
Marker for diagnosis and follow-up
Same day
MRI of the liver
PRETEXT staging, vessels
One to two days
Chest CT
Lung metastases
Same visit
Needle biopsy
Confirms type, guides treatment
Results in three to five days
We complete these tests within a week of arrival.
Hearing and kidneys before treatment
A baseline hearing test, kidney function and a heart ultrasound give reference values before cisplatin starts, and every later test is compared with them. We repeat them during treatment.
PRETEXT staging
PRETEXT groups, by liver sections free of tumor
Group
Tumor-free sections
Surgical meaning
PRETEXT I
Three adjoining sections free
Small resection
PRETEXT II
Two adjoining sections free
Standard resection
PRETEXT III
One section free, or two not adjoining
Extended or central resection
PRETEXT IV
No section free
Transplant assessment
Annotation factors
The 2017 update of PRETEXT adds letters for spread into the hepatic veins or vena cava, V, the portal vein, P, outside the liver, E, several separate tumors, F, rupture, R, and metastases, M (Towbin and colleagues, Pediatric Radiology, 2018). Each letter changes the risk group and the operation.
Staging is repeated after chemotherapy, called POST-TEXT, and the surgeon plans the operation on that second scan.
1
Three-dimensional planning
Our radiologists and surgeons build a three-dimensional model of the liver from MRI, showing the tumor, veins and the volume of liver that will remain.
Risk groups
1
One international system
The Children's Hepatic tumors International Collaboration pooled 1,605 children from eight trials over 25 years and created four risk groups, very low, low, intermediate and high (Meyers and colleagues, Lancet Oncology, 2017).
The groups combine PRETEXT, age, AFP level and the annotation factors. Age three years or older, AFP of 100 or below, metastases and major vessel involvement point to higher risk.
2
What changes with risk
Very low risk tumors can be removed first with little or no chemotherapy. Higher risk tumors need longer and more intensive chemotherapy before surgery.
Risk and treatment
How risk group shapes treatment
Risk group
Chemotherapy
Surgery
Very low
None or short course
Resection at diagnosis
Low and intermediate
Cisplatin based, four to six cycles
Resection after chemotherapy
High, metastases
Intensive combinations
Resection or transplant, lung surgery if needed
Why one team plans everything
Hepatoblastoma rewards careful planning.
The operation decides cure, and its timing, extent and type are decided before chemotherapy even starts, because a child whose tumor may need a transplant must be referred to a transplant team early, while chemotherapy is still shrinking the tumor, and not after a risky partial operation has failed. A review of the world experience found long-term survival near 80 percent after a planned primary transplant and near 30 percent after a rescue transplant for tumor left behind or regrowth after resection (Otte and colleagues, Pediatric Transplantation, 2005). At Biruni Hospital, pediatric oncologists, liver surgeons, transplant surgeons and radiologists review each child together at the tumor board, from the first scan.
One plan, from day one.
Chemotherapy before surgery
Chemotherapy before surgery shrinks the tumor, pulls it away from the large veins and makes complete removal safer.
Standard risk results
Cisplatin alone against cisplatin with doxorubicin, 255 children
Outcome
Cisplatin alone
Cisplatin and doxorubicin
Complete removal
95 percent
93 percent
Three-year survival
95 percent
93 percent
Severe side effects
20.6 percent
74.4 percent
How it is given
Cisplatin runs through a drip every two weeks, four cycles before surgery and two after, through a central line placed under anesthesia at the start (Perilongo and colleagues, 2009). Higher risk tumors receive added drugs such as doxorubicin, carboplatin or etoposide, and AFP falls with each cycle as the tumor responds.
Protecting your child's hearing
1
Sodium thiosulfate
Given into a vein six hours after each cisplatin dose, sodium thiosulfate binds cisplatin in the inner ear and protects hearing.
In the SIOPEL 6 trial of 109 children, hearing loss fell from 63 to 33 percent, and three-year survival was 98 percent with sodium thiosulfate against 92 percent without it.
Hearing tests before each cycle find early loss in the high tones, and the doctors adjust treatment. Children with hearing loss receive hearing aids early, so that speech develops normally (Brock and colleagues, 2018).
Does sodium thiosulfate reduce the effect of chemotherapy?
In standard risk hepatoblastoma, the trial showed no loss of tumor control. For tumors with metastases, the evidence is thinner, and we explain the reasoning before deciding together with the family.
Liver resection
Types of liver resection for hepatoblastoma
Operation
What is removed
Used for
Segmentectomy
One or two segments
PRETEXT I
Hemihepatectomy
Right or left half
PRETEXT II
Trisectionectomy
Three of four sections
PRETEXT III
Central resection
Middle sections
Central tumors
1
The aim
Removal of the whole tumor with a margin of normal liver, confirmed by the pathologist under the microscope.
Ultrasound during surgery maps the veins and searches the rest of the liver for small tumors that scans missed.
The blood supply is divided first.
The liver is cut with devices that seal small vessels, keeping blood loss low.
The operation takes four to six hours. The remaining liver grows back to normal size within weeks, so children live a normal life with part of the liver removed.
When resection is risky
Tumors that remain close to all three hepatic veins or the portal vein split after chemotherapy need very large or complex operations. In these cases we compare extreme resection with transplant openly, and the safer route wins.
No child should undergo a hazardous partial operation that leaves tumor behind. Rescue transplant results are far worse than planned transplant results.
Transplant for tumors that cannot be resected
Primary against rescue transplant for hepatoblastoma
Approach
When
Long-term survival
Primary transplant
PRETEXT IV or central tumor after chemotherapy
Near 80 percent
Rescue transplant
Tumor left behind or regrowth after resection
Near 30 percent
Living donor
A parent donates part of the liver
Planned timing, no waiting list
Transplant replaces the whole liver, removing every tumor cell within it. A living donor, a parent in most cases, gives the left part of the liver, and the operation is timed right after the last chemotherapy cycle. Our pediatric liver transplant page describes the donor evaluation, the operation and life afterward.
Chemotherapy continues for two cycles after the transplant, with drugs to prevent rejection adjusted for the kidneys.
Recovery after surgery
1
Intensive care first
Children spend one to two days in pediatric intensive care, with an epidural or a pain pump, then move to the ward with a parent in the room.
A typical recovery after resection
Stage
Timing
What happens
First drinks and food
Day one to three
Diet builds up
Walking and play
From day two
Physiotherapy support
Drain and line care
Day three to five
Drains removed
Discharge
Day seven to ten
Eating, walking, comfortable
Liver blood tests rise briefly after surgery and return to normal as the remaining liver grows. Chemotherapy restarts three to four weeks after the operation, once the wound has healed.
Lung metastases
One in six children has spread to the lungs at diagnosis. Chemotherapy clears most of these nodules, and nodules that remain are removed by keyhole or open lung surgery, before or after the liver operation, with the goal of cure.
Lung metastases do not rule out cure. Many children with metastatic hepatoblastoma become long-term survivors.
Follow-up and AFP
Follow-up after hepatoblastoma treatment
Test
How often
Purpose
AFP
Monthly in year one, then every two to three months
Earliest sign of return
Abdominal ultrasound
Every three months in year one
Liver and remaining tumor bed
Chest imaging
Every three to six months at first
Lung metastases
Hearing, kidney, heart tests
Yearly
Late effects of chemotherapy
1
A rising AFP
A steady rise in AFP after treatment calls for imaging at once, even when the child is well.
Most relapses appear in the first two years. Follow-up continues to adulthood for growth, hearing, kidney function and school progress.
Results can be shared from home, and our team reviews each AFP within days.
Life after treatment
Children return to nursery and school.
After hepatoblastoma, the remaining liver works as before, children eat the same food as their siblings, play sport and receive their missed vaccinations once the oncology team agrees, while children who had a transplant take daily anti-rejection medicine and come for regular blood tests, and every child gets yearly hearing checks with hearing aids or school support whenever the tests show high tone loss. Parents carry the weight of the whole treatment, and our psychologists and social workers support them through it.
Childhood goes on after cancer.
For families from abroad
1
Length of stay
Treatment takes four to six months in total. Chemotherapy cycles run every two weeks, surgery comes after the fourth cycle, and the two final cycles follow. Fitness to fly between phases depends on blood counts and the wound.
Send AFP results, MRI or CT images with reports, the biopsy report and slides if available, and any chemotherapy given so far. The tumor board reviews them and proposes a plan before you travel.
Ways families arrange treatment
Plan
Where chemotherapy is given
Where surgery is done
All in Istanbul
Biruni Hospital
Biruni Hospital
Shared care
Home country, our protocol
Biruni Hospital
Surgery only
Home country
Biruni Hospital, with two to three weeks stay
2
After you return home
AFP and scans continue with your local team, and we review every result by video and message.
One coordinator from the international patients office handles arrangements from the first message through discharge and answers the same WhatsApp number after you return. The office works in English, Arabic, French, Russian, Serbian, Romanian and Spanish and books interpreters for other languages, and it arranges the visa invitation letter, airport transfers, daily transport and accommodation near the hospital. Halal, vegetarian and diabetic meals, a prayer room on site, and a female physician on request wherever the rota allows.
3
Families staying for months
Apartments near the hospital and a parent bed in every admission.
For a living donor transplant, the donor parent needs a full evaluation before travel, which we start by sending the test list.
Cost
The quote follows the treatment plan the tumor board proposes, which depends on risk group and the operation needed.
Transplant quotes include the donor evaluation, the donor's operation and hospital stay, and the first months of anti-rejection medicine for the child.
1
Written quote
Sent before you travel.
Compare quotes for the same plan, and check that hearing protection and follow-up are part of it.
2
Shared care
When chemotherapy is given at home, the quote covers surgery and the hospital stay.
References
Perilongo G, Maibach R, Shafford E, et al. Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma. N Engl J Med. 2009;361(17):1662-1670.
Brock PR, Maibach R, Childs M, et al. Sodium thiosulfate for protection from cisplatin-induced hearing loss. N Engl J Med. 2018;378(25):2376-2385.
Meyers RL, Maibach R, Hiyama E, et al. Risk-stratified staging in paediatric hepatoblastoma, a unified analysis from the Children's Hepatic tumors International Collaboration. Lancet Oncol. 2017;18(1):122-131.
Towbin AJ, Meyers RL, Woodley H, et al. 2017 PRETEXT, radiologic staging system for primary hepatic malignancies of childhood revised for the Paediatric Hepatic International Tumour Trial (PHITT). Pediatr Radiol. 2018;48(4):536-554.
Otte JB, de Ville de Goyet J, Reding R. Liver transplantation for hepatoblastoma, indications and contraindications in the modern era. Pediatr Transplant. 2005;9(5):557-565.
Editor's note
Written by the Biruni Hospital medical editorial team. Reviewed by Assoc. Prof. Dr. Elif ACAR ARSLAN, Pediatric Neurology.
