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Hepatoblastoma in infancy and childhood: a clinical and pathological study of 32 cases
1Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.
Insights
Hepatoblastoma, a pediatric liver cancer, often presents as an abdominal mass in children under three. Complete surgical removal of the tumor is crucial for survival in these young patients.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Diseases
- Cancer Research
Background:
- Hepatoblastoma is a rare but aggressive childhood liver cancer.
- Early diagnosis and treatment are critical for improving outcomes.
Purpose of the Study:
- To analyze the clinical characteristics, treatment, and survival rates of hepatoblastoma patients.
- To identify key factors influencing patient prognosis.
Main Methods:
- Retrospective review of 32 pediatric hepatoblastoma cases diagnosed between 1959 and 1988.
- Analysis of patient demographics, clinical presentation, laboratory findings, tumor histology, treatment, and survival data.
Main Results:
- The mean age at diagnosis was 1 year and 8 months, with 87.5% of patients under three.
- Common signs included abdominal mass (71.9%) and distention (62.5%).
- Elevated alphafetoprotein (92.3%) and thrombocytosis (64.3%) were frequent laboratory findings.
- The two-year survival rate was 21.4%, with complete tumor resection being the only factor associated with survival.
Conclusions:
- Hepatoblastoma predominantly affects infants and young children.
- Complete surgical resection is the most critical factor for achieving long-term survival in hepatoblastoma patients.
Abstract:
During the twenty-nine-year period from June 1959 to October 1988, 32 infants and children with histology-proved hepatoblastoma were seen at the Department of Pediatrics, National Taiwan University Hospital (NTUH). The age at diagnosis ranged from four days to six years, with a mean of one year and eight months. Most of the patients (87.5%) were under the age of three. There was no sex predilection (16 males, 16 females). The most common presenting signs were an upper abdominal mass (71.9%) and/or abdominal distention (62.5%). Physically, all the patients revealed hepatomegaly, with or without mass. Laboratory abnormalities included primarily thrombocytosis (64.3%), elevated serum aspartate aminotransferase (75.0%), serum alanine aminotransferase (62.5%), cholesterol (78.3%) and alphafetoprotein (92.3%). According to the classification of Ishak and Glunz, 56.3% of these belonged to the epithelial type; the others belonged to the mixed type. During the follow-up period, 6 patients with complete resection of the tumor remained alive from 2 years to 17 years and 4 months. Twenty-two patients died from 8 days to 16 months after diagnosis. Four patients were lost to follow-up. The two-year survival rate was 21.4% (6/28). Complete resection of the tumor was the key treatment for achieving survival.