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Tumor rupture in hepatoblastoma: A high risk factor?
Morgane Pondrom1, Daniele Pariente2, Brenda Mallon1
1Gustave Roussy, Department of Children and Adolescents Oncology, Université Paris-Saclay, Villejuif, France.
Pediatric Blood & Cancer
|July 4, 2020
Summary
Hepatoblastoma tumor rupture indicates a poor prognosis, increasing the risk of peritoneal spread. However, this high-risk factor should not prevent liver transplantation for affected children.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Hepatobiliary Diseases
Background:
- Hepatoblastoma tumor rupture is a high-risk factor in the SIOPEL 3/4 protocol.
- Outcomes for children with hepatoblastoma and tumor rupture are not well-documented.
Purpose of the Study:
- To investigate the clinical outcomes of hepatoblastoma patients experiencing tumor rupture.
- To evaluate the impact of tumor rupture on prognosis and treatment strategies.
Main Methods:
- Retrospective review of 150 hepatoblastoma cases (2000-2014) in France.
- Radiological assessment for signs of tumor rupture (peritoneal effusion, nodules, subcapsular hematoma).
- Analysis of clinical data, treatment regimens (chemotherapy, surgery, liver transplant), and survival rates.
Main Results:
- 24 patients (16%) had radiological signs of tumor rupture.
- Intraperitoneal rupture occurred in 17 patients, often associated with subcapsular hematoma.
- Complete remission was achieved in 68% of patients; 3-year event-free survival was 49.6% and overall survival was 68.2%.
- Tumor rupture predicted peritoneal progression/relapse.
Conclusions:
- Hepatoblastoma tumor rupture is associated with a poor prognosis and increased risk of peritoneal disease.
- Liver transplantation remains a viable option and should not be contraindicated solely based on tumor rupture.
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