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
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.
What hepatoblastoma is
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.
| 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
Signs and first tests
| 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?
| 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 |
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
| 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
Staging is repeated after chemotherapy, called POST-TEXT, and the surgeon plans the operation on that second scan.
Risk groups
Risk and 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
| 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
Protecting your child's 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.
Does sodium thiosulfate reduce the effect of chemotherapy?
Liver resection
| 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 |
- 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.
When resection is risky
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
| 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 |
Chemotherapy continues for two cycles after the transplant, with drugs to prevent rejection adjusted for the kidneys.
Recovery after surgery
| 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 |
Lung metastases
Lung metastases do not rule out cure. Many children with metastatic hepatoblastoma become long-term survivors.
Follow-up and AFP
| 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 |
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
| 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 |
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.
| Item | Included | Priced separately |
|---|---|---|
| Surgery | Liver surgeon, anesthesia, operating room, pathology | Lung surgery, transplant |
| Hospital | Intensive care and ward stay as planned | Extra days |
| Chemotherapy | Cycles in the plan, central line | Extra cycles or drugs |
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 Prof. Dr. Mustafa Atilla NURSOY, Pediatric Allergy and Immunology.
Medically reviewed by
Prof. Dr. Mustafa Atilla NURSOY
Pediatric Allergy and Immunology
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