Clinical

Intensive therapy improves survival in high-risk hepatoblastoma

Last Updated: 2010-04-21 20:24:28 -0400 (Reuters Health)

NEW YORK (Reuters Health) - Treatment with dose-intensive multi-agent chemotherapy and surgery improves survival in patients with high-risk hepatoblastoma, according to results of the SIOPEL-3HR Study.

In the original SIOPEL study, reported in 2000, all patients received preoperative chemotherapy (PLADO; cisplatin plus doxorubicin) and delayed surgery, which led to improved overall and event-free survival compared to previous reports. However, the prognosis was still unsatisfactory in children with tumor involving all four liver sections or metastasis.

In the current study, Dr. Jozsef Zsiros, of the University of Amsterdam, the Netherlands, and colleagues assessed the efficacy of a newly designed preoperative chemotherapy regimen in children with high-risk hepatoblastoma.

They defined high risk based on features of the disease at diagnosis: tumor in all liver sections (PRETEXT-IV), or presence of distant metastases (M+), or vascular invasion of the portal vein (P+), three hepatic veins (V+), or intra-abdominal extrahepatic extension (E+), or alpha-fetoprotein less than 100 ng/mL.

The patients received alternating cycles of cisplatin and carboplatin plus doxorubicin and delayed tumor resection. Seven cycles were performed preoperatively, and three were performed postoperatively.

Results of the study were published online April 20th in the Journal of Clinical Oncology.

Among 151 in the study, 118 patients (78.7%) achieved a partial response to chemotherapy. Among the 69 patients with initial lung metastases, 36 (52.2%) achieved complete remission with chemotherapy alone.

Complete resection of the liver was achieved in 115 patients (76.2%), either by partial hepatectomy (n = 84) or by liver transplantation (n = 31). In 106 patients (70.2%), complete resection of all tumor lesions (including metastases) was achieved.

The tumor was removed by liver resection in 31 of 74 patients with initial PRETEXT-IV tumor. Another 26 of these patients underwent liver transplantation, resulting in a complete liver tumor resection rate of 77.0% for this group.

At 3 years, event-free survival and overall survival for the whole group was 65% and 69%, respectively. For patients with PRETEXT-IV tumor and those with metastasis, event-free survival was 68% and 56%, respectively, and overall survival was 69% and 62%, respectively.

The SIOPEL group is currently studying the efficacy of a regimen with high cisplatin dose-intensity in high-risk patients, as well as the efficacy of irinotecan in patients with recurrent disease. "These approaches might be part of future therapeutic strategies against advanced hepatoblastoma," the researchers say.

J Clin Oncol 2010.