Management outcome of Hepatoblastoma in Srinagarind Hospital

: 5-year review
Wichien Sirithanaphol, MD

Introduction
Hepatoblastoma (HB) is the most common paediatric liver malignancy.
It is quite rare. Accounting for only 0.9% of all pediatric cancers.1

The diagnosis of hepatoblastoma is usually difficult until clinical manifestations caused by the tumor appear.
1. Miller RW, Young JL Jr, Novakovic B (1995) Childhood cancer. Cancer 75:395405

Introduction
The number of patients with hepatoblastoma who have undergone complete resection is not large.
Only 3050% of tumors are resectable at diagnosis.
So in most cases, preoperative chemotherapy is indicated. Can reduce the size of tumors, making them resectable by conventional surgery.2
2. Schnater JM, Aronson DC, Plaschkes J, et al (2002) Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL-1). Cancer 94:11111120

Introduction
Multimodal treatment, including chemotherapy and surgical treatment, have led to marked improvements in therapeutic effects for this disease.
The purpose of this study was to retrospectively evaluate the results of surgical treatment with perioperative chemotherapy in Srinagarind Hospital over 5-year period.

Patient & Method

Research methodology
Retrospective review : Chart review
At Srinagarind hospital, university hospital in Khon-Kaen Period of study : 1 Jan 2004 - 31 Dec 2008 Retrieve data from ICD10 code
Hepatoblastoma ICD code : C222

Research methodology
14 patients with Hepatoblastoma
Patient characteristics, perioperative data, chemotherapy regimen and pathological results were recorded.

Staging
Hepatoblastoma staging
Stage1 : No metastasis, tumor completely resected Stage2 : No metastasis, tumor grossly resected with microscopic residual disease (positive margin) or tumor rupture, or tumor spill at the time of surgery Stage3 : No distant metastasis, tumor unresectable or resected with gross residual tumor or positive lymph nodes Stage4 : Distant metastasis regardless of the extent of liver involvement

Chemotherapy regimen
Chemotherapy regimen : IPA (HB89 protocol) regimen
Ifosfamide 1 gm/m2 iv in 8 hr with Mesna day1,2,3 Cisplatin 100 mg/m2 iv in 1 hr with Mannitol day4 Adriamycin 40 mg/m2 iv push day5

Repeat cycle q 21 days

Stage1 : 3IPA Stage2 : 4IPA Stage3-4 : 2-4IPA 2nd resection 2IPA

Results

Demographic Data (14 patients)

Male Female Mean age

Underlying

9 (64%) 5 (36%) 2.1 yr (2 mo 13 yr)

1 Epilepsy 1 Thalssemia 1 G-6PD deficiency
4 (3-5.2 g/dl)

Albumin

Clinical presentation
Abdominal pain 1 pt (7%) (Traumatic ruptured tumor)

Paraparesis 1 pt (7%) (SC metastasis)

Abdominal mass 12 pt (86%) (Asymptomatic)

Imaging
All patients : Preoperative abdominal CT scan
Tumor location
Right lobe

14 pt
6 pt (42%)

Left lobe
Both lobe

4 pt (29%)
4 pt (29%)

Imaging
All patients : Preoperative abdominal CT scan
Tumor calcification
Yes

14 pt
13 pt (93%)

No

1 pt (7%)

Staging
Stage 4 : 4 pt (29%)

Stage 1 : 0 pt (0%)
Stage 2 : 1 pt (7%)

Stage 3 : 9 pt (64%)

Staging
Stage 4 : 4 pt (29%)

Unresectable 13/14 pt (93%)

Stage 3 : 9 pt (64%)

Staging
Stage 4 : 4 pt (29%)

Lung metastasis 3 pt

SC metastasis 1 pt

Management
Hepatoblastoma 14

Operation

12

No operation 2

Management
Hepatoblastoma 14

Operation

12

No operation 2

Management
Hepatoblastoma 14

Operation

12

No operation 2

SC metastasis 1

Adjuvant CMT

Progression (unresectable disease) 1

Management
Hepatoblastoma 14

Operation

12

No operation 2

Management
Hepatoblastoma 14

Operation

12

No operation 2

Adjuvant CMT 11/12

Hepatic resection 10

Liver biopsy 2

Management

Procedure

Patients

Right hepatectomy
Left hepatectomy Segmentectomy

6
3 1

Hepatic resection 10

Management
Operative time (min)
151 (95-180 min)

Blood loss (ml)

Blood transfusion Intraoperative complication Postoperative complication Drain removal (day) Hospital stay (day)

187 (60-500 ml)

LPB 1.6 (0-2 dose) 1 massive bleeding 1 chylous ascitis 7.2 (4-16 day) 11.7 (6-16 day)

Hepatic resection 10

Size of tumor
At time of diagnosis 390 cm3

Volume = (A*B*C)/2

After Neoadjuvant CMT 130 cm3

Progression (unresectable disease) 1

Regression rate

66%

Tumor marker : AFP

350000 300000 250000 200000 150000 100000 50000 0 AFP diagnosis 34409 AFP post CMT 18482 AFP post op 296990

Pathology
Hepatoblastoma 14

Operation

12

No operation 2

Adjuvant CMT 11/12

Hepatic resection

10

Liver biopsy 2 Hepatoblastoma

SC metastasis 1
Metastatic Hepatoblastoma

Progression 1 No pathology

8 Free margin 2 Not free

Outcome
No recurrent 5 (35%) Progression or Palliative 8 (56%)

Hepatoblastoma 14

Loss F/U 1 (7%)

Mean Follow up Time = 22 months

Discussion
Long-term survival is expected in infants and children with hepatoblastoma, if a complete resection is possible.
If more than 3-4 segments of the liver are occupied by the tumor, or has distant metastases at the initial presentation A complete resection is considered to be impossible.
Resection after neoadjuvant chemotherapy became possible.

Discussion
In this study, most patients present with advanced stage
Perioperative chemotherapy improved the resection rate

Hepatic resection is quite safe and effectiveness

No motality 2 morbidity
1 Massive bleeding 1 Chylous ascitis : successful conservative treatment

Conclusion
The combination of surgery and (neo) adjuvant chemotherapy has improved patient outcomes
However, this review involved only a small number of patients, further study should be performed for definitive conclusion

Thank You