It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.
Child ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Radiotherapy*

In hepatoblastoma, encouraging cure rates have been achieved with recent advances in chemotherapy and surgical techniques. The aim of this study is to evaluate the role of combined therapeutic modalities and surgical resection in hepatoblastoma. Fifteen cases of hepatoblastoma were treated from January 1993 to August 2000. Six patients had resectable tumors at initial diagnosis. All underwent surgical resection and in four patients postoperative adjuvant chemotherapy was needed. Nine out of 15 patients had unresectbale tumors at initial diagnosis, and preoperative chemotherapy was applied. There was one operative mortality and 14 patients showed good prognosis after surgery. Although various treatment modalities should be combined for the unresectable hepatoblastoma, surgical resection remains the major curative procedure.
Chemotherapy, Adjuvant ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Mortality ; Prognosis

Hepatoblastoma is the most common primary malignant hepatic tumor in infancy and childhood. But congenital hepatoblastomas are extremely rare and show distinctive, but important features when compared with tumors diagnosed in children beyond the neonatal age. They have different clinical presentations, higher incidence of pure fetal histology, significant risk for systemic metastasis, and worse outcome. The treatment of congenital hepatoblastoma should be the same as in older children, i.e., primary chemotherapy with delayed resection. We report a case of congenital hepatoblastoma in a 29-day-old boy who was known to have a well-defined ovoid hypoechoic mass at liver demonstrated by fetal sonography.
Child ; Drug Therapy ; Hepatoblastoma* ; Humans ; Incidence ; Liver ; Male ; Neoplasm Metastasis

Oxidative stress appears to be implicated in the pathogenesis of various diseases including alcoholic liver injury. In this study we investigated the mechanism of apoptosis induced by tert-butyl hydroperoxide (TBHP) in HepG2 human hepatoblastoma cells. Treatment with TBHP significantly reduced glutathione content and glutathione reductase activity, and increased glutathione peroxidase activity, indicating that TBHP induced oxidative stress in the HepG2 cells. TBHP also induced reduction of cell viability and DNA fragmentation, a hallmark of apoptosis, in a dose-dependent manner. In addition, TBHP induced a sustained increase in intracellular Ca2+ concentration, which was completely prevented by the extracellular Ca2+ chelation with EGTA. TBHP also induced Mn2+ influx. These results indicate that the intracellular Ca2+ increase by TBHP is exclusively due to Ca2+ influx from the extracellular site. Treatment with either an extracellular (EGTA) or an intracellular Ca2+ chelator (BAPTA/AM) significantly suppressed the TBHP-induced apoptosis. Taken together, these results suggest that TBHP induced the apoptotic cell death in the HepG2 cells and that Ca2+ influx may play an important role in the apoptosis induced by TBHP.
Apoptosis/drug effects* ; Calcium Signaling/drug effects* ; Chelating Agents/pharmacology ; Egtazic Acid/pharmacology ; Egtazic Acid/analogs & derivatives ; Hepatoblastoma/pathology* ; Hepatoblastoma/metabolism ; Hepatoblastoma/drug therapy ; Human ; Manganese/metabolism ; Oxidative Stress/drug effects ; Tumor Cells, Cultured ; tert-Butylhydroperoxide/pharmacology*

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage, but improved resectability and survival is being reported with the help of neoadjuvant chemotherapy. Twenty biopsy proven hepatoblastoma patients were diagnosed and managed during the period between January, 1987 and June, 1995. Median age at diagnosis was 13 months (2mo~7yr 10mo) with M : F = 13 : 7. The histologic profile included 13 epithelial (5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial types. Chemotherapy effectively reduced the tumor volume with statistical significance (p=0.008) and was able to convert 7 out of 9 initially unresectable cases (78%) to resectable ones. Fourteen operations were done, 12 radical and 2 palliative with or without adjuvant chemotherapy. The whole population was followed with a median duration of 33 months and the median survival in the whole group was 26 months. The curative resection group displayed a 5 year survival rate of 61.1% and none in the non-curative group survived for more than 13 months (p=0.0001). In univariate analysis for prognostic factors, large tumor size at diagnosis and absence of thrombocytopenia was associated with poor survival, but these differences were not statistically significant. Pure fetal histology was not associated with better prognosis and this may be due to a different histologic classification. In this new era of neoadjuvant chemotherapy, the optimal management strategy for hepatoblastoma is still debated with radical surgical resection at earliest possible time being the final goal. For now, individualized approach appears to be the choice.
Biopsy ; Chemotherapy, Adjuvant ; Classification ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Prognosis* ; Survival Rate ; Thrombocytopenia ; Tumor Burden

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage. Complete surgical resection after chemotherapy is the definitive treatment for hepatoblastoma. Liver transplantation should be considered for children who have unresectable hepatoblastoma. We report a case of a 18-month-old boy with unresectable hepatoblastoma who had transplantation with pre- and post-operative chemotherapy.
Child ; Drug Therapy ; Hepatoblastoma* ; Humans ; Infant ; Liver Transplantation* ; Liver* ; Living Donors* ; Male

PURPOSE: The purpose of this study was to analyze the CT findings of hepatoblastoma before and afterchemotherapy, and to compare them with surgical and pathologic features. MATERIALS AND METHODS: Twelvehepatoblastoma patients underwent chemotherapy prior to surgery; in all cases, CT scanning was performed beforeand after chemotherapy. We reviewed the findings with special attention to changes in tumor volume, the extent andpattern of contrast enhancement, the extent of low-attenuation area in the tumor, the presence of a septum, andcalcification or ossification within the mass before and after chemotherapy. Post-chemotherapy CT findings werecompared with operative and pathologic findings. RESULTS: After chemotherapy, the volume of the tumor massdecreased in all patients, and the extent of involved segments decreased in nine(75%), the non-enhancing areawithin the mass, on the other hand, increased in nine (75%). On pre-chemotherapy CT, calcifications were detectedin seven patients(58%), and on post-chemotherapy CT, in nine (75%); the extent of calcification increased in sevenpatients. On the basis of CT findings, viable tumor and necrosis areas could not be distinguished. Massivecalcification or an osteoid mixed with loose connective tissue was noted in the mesenchymal component of thetumor; the whirling pattern of enhancement within the area of low density asen on CT scanning corresponded toosteoid mixed with loose connective tissue, which contained rich blood vessels. CONCLUSION: We describe the CTfindings of hepatoblastoma both before and after chemotherapy, highlighting the changes which occurred. Anunderstanding of these changes is helpful for the proper management of this condition.
Blood Vessels ; Connective Tissue ; Drug Therapy* ; Hand ; Hepatoblastoma* ; Humans ; Necrosis ; Tomography, X-Ray Computed ; Tumor Burden

Hepatoblastoma is an uncommon malignant tumor of the liver in children, moreover its intracaval extension to the right atrium is extremely rare. Current data indicate that combined hepatectomy and resection of intraatrial extension is necessary for cure. However 40% of the patients present with tumor that would make primary complete resection impossible or extremely hazardous. In these case preoperative chemotherapy is recommanded. We recently experienced a rare case of hepatoblastoma which metastasized to lung and to right atrium via inferior vena cava. We report this case with brief review of literature.
Child ; Drug Therapy ; Heart Atria ; Hepatectomy ; Hepatoblastoma ; Humans ; Liver ; Lung ; Vena Cava, Inferior

Hepatoblastoma is the most common primary malignant liver tumor encountered during childhood. Complete surgical resection is the most important factor in predicting which children would achieve cure. Some chemotherapy regimens are effective in reducing tumor size, often rendering initially unresectable lesions amenable to complete surgical resection, and dramatically improving disease-free survival. Liver transplantation provides an additional treatment option for patients whose tumor remains unresectable even after preoperative chemotherapy. We report a case of unresectable hepatoblastoma in a child who underwent living related liver transplantation with pre and post-operative chemotherapy.
Child* ; Disease-Free Survival ; Drug Therapy ; Hepatoblastoma* ; Humans ; Liver Transplantation* ; Liver* ; Living Donors*

Spontaneous rupture with internal bleeding of solid tumors has rarely been described at the time of diagnosis or during chemotherapy. This rare event must be regarded as a life threatening condition. In these emergency situations, control of hemorrhage, which is life-saving, can be achieved by transcatheter arterial embolization (TAE) and/or surgical resection. This report describes two infants presenting with acute hemorrhagic shock due to spontaneous tumor rupture of hepatoblastoma and neuroblastoma during chemotherapy. TAE successfully arrested the tumor bleeding and a visibly reduced the tumor size in both children. Spontaneous rupture of solid tumors occur infrequently in children, but is a life threatening situation. Careful monitoring for the occurrence of this rare event especially in very young children presenting with a large tumor mass.
Child ; Diagnosis ; Drug Therapy ; Emergencies ; Hemorrhage ; Hepatoblastoma ; Humans ; Infant ; Neuroblastoma ; Rupture ; Rupture, Spontaneous ; Shock, Hemorrhagic