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Primitive malignant nonepithelial hepatic tumors in children
A Babin-Boilletot1, F Flamant, M J Terrier-Lacombe
1Service d'Onco-Hematologie, Centre Hospitalier Régional, Strasbourg, France.
Medical and Pediatric Oncology
|January 1, 1993
Summary
Primary pediatric liver cancers are rare non-epithelial tumors. Complete surgical resection is crucial for cure, but outcomes remain challenging, with a 2-year disease-free survival of 37%.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Malignancies
- Surgical Oncology
Background:
- Primary intrahepatic malignant nonepithelial tumors in children are exceptionally rare, comprising only 2% of pediatric malignant mesenchymatous tumors.
- Clinical presentation, radiological findings, and histological classifications of these tumors are often ambiguous, complicating diagnosis and treatment planning.
Purpose of the Study:
- To review the clinical presentation, diagnostic features, and management strategies for primary intrahepatic malignant nonepithelial tumors in pediatric patients.
- To evaluate the efficacy of different treatment modalities, including surgery, chemotherapy, radiotherapy, and liver transplantation.
- To determine the prognostic factors and survival rates associated with these rare pediatric liver cancers.
Main Methods:
- Retrospective analysis of pediatric patients diagnosed with primary intrahepatic malignant nonepithelial tumors.
- Review of clinical data, imaging studies, histopathological reports, and treatment records.
- Statistical analysis of treatment outcomes and disease-free survival rates.
Main Results:
- Complete surgical resection is the cornerstone of treatment, either as an initial approach for localized tumors or following neoadjuvant chemotherapy.
- Radiotherapy demonstrated limited efficacy in improving outcomes.
- High-dose chemotherapy and liver transplantation were considered for refractory cases, with an overall 2-year disease-free survival rate of 37% for the entire cohort.
Conclusions:
- Complete tumor resection is essential but not sufficient for cure in pediatric intrahepatic malignant nonepithelial tumors.
- Multimodal therapy, including chemotherapy and potentially liver transplantation, may be necessary for managing resistant disease.
- The overall prognosis remains guarded, highlighting the need for further research into novel therapeutic strategies for these rare pediatric liver malignancies.