Abstract

Hepatoblastoma (HB) is a malignancy of the liver in childhood and comprises 1% of all pediatric malignancies. After neuroblastoma and Wilms tumor, HB is the third most common intraabdominal neoplasms in children. Ultimate goal of treatment for HB is total tumor resection. Japanese Study Group for Pediatric Liver Tumor (JPLT) has been established since 1991. In JPLT-2 clinical trials from 1998, the staging system PRETEXT", which is based on the extent of the tumor at the initial untreated status based on the Couinaud sections, is used for determining resectability of the tumor. Disease with PRETEXT I to III is commonly adapted preoperative chemotherapy, whose key drug is cisplatin, followed by hepatectomy. Patients with PRETEXT IV tumors, multifocal tumors and tumorsinvading major vessels of the liver are candidates for livertransplantation. The existence of pulmonary metastasis is a predictor of poor prognosis and metastasectomy is advocated. The 5-year overall survival in HB with PRETEXT I to III was over 80% in JPLT-2. Therefore, multimodal treatment with anatomical liver resection is promising for affected children without metastasis. The treatment for patients with PRETEXT IV or metastasis is still challenging.