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Thirty years of experience with pediatric primary malignant liver tumors
Insights
Primary hepatic malignancy in children is rare. Complete surgical resection offers the best chance for long-term survival, particularly for hepatoblastoma and fibrolamellar carcinoma.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Surgery
- Medical Imaging
Background:
- Primary hepatic malignancy in children is a rare but serious condition.
- Presentation varies from asymptomatic to advanced disease, with palpable abdominal mass being common.
Purpose of the Study:
- To review the clinical presentation, diagnostic methods, and outcomes of primary hepatic malignancy in children.
- To evaluate the effectiveness of different treatment modalities and identify prognostic factors.
Main Methods:
- Retrospective analysis of 48 pediatric patients diagnosed with primary hepatic malignancy between 1950 and the study period.
- Review of clinical data, diagnostic imaging (hepatic angiography, computed tomography scans), tumor markers (alpha-fetoprotein), and treatment outcomes.
- Histopathological classification of tumors.
Main Results:
- Hepatoblastoma was the most common histology (39/48).
- Complete surgical resection resulted in 11 long-term survivors (6 months to 23 years).
- Fibrolamellar carcinoma showed a favorable prognosis with resection (3/4 survivors). Hepatocellular carcinoma and mesenchymoma had poor outcomes.
Conclusions:
- Complete surgical resection is crucial for curative treatment of pediatric primary hepatic malignancy.
- Hepatoblastoma and fibrolamellar carcinoma appear to have better prognoses following resection.
- The role of adjuvant therapies requires further investigation.
Abstract:
Since 1950, 48 infants and children from 10 weeks to 16 years of age presented with primary hepatic malignancy. Signs and symptoms ranged from asymptomatic to those of malignant disease. All patients had a palpable abdominal mass. Jaundice was seen in five patients, four of whom had preexisting cirrhosis. Three male children had evidence of precocious puberty. Whereas liver function tests were usually normal, alpha-fetoprotein levels, when elevated, proved useful diagnostically and as a tumor marker in follow-up. Hepatic angiography and computed tomography (CT) scans have provided the most valuable preoperative assessment of hepatic architecture. Sixteen infants and children underwent resection for cure. Eleven of these patients are alive and disease free 6 months to 23 years later. Six additional patients had incomplete resection with subsequent radiotherapy and/or chemotherapy; only one such patient is disease-free past 3 years. Twenty-six tumors could only be biopsied; most of these patients died within 12 months regardless of what treatment they received. The histology was hepatoblastoma in 39 patients, hepatocellular carcinoma in 4, fibrolamellar carcinoma in 4, and malignant mesenchymal tumor (mesenchymoma) in 1. The patients with hepatocellular carcinoma and mesenchymoma all died. Three of four patients with fibrolamellar carcinoma are alive and disease-free following resection up to 3 years; this histology seems favorable. The other survivors had hepatoblastoma. The role of adjunctive chemotherapy and/or radiotherapy has not yet been determined.