Primitive malignant nonepithelial hepatic tumors in children

Primary intrahepatic malignant nonepithelial tumors are very rare in children and account for 2% of all malignant mesenchymatous tumors under the age of 15 years. Clinical presentation, radiologic features, and histologic types are not unequivocal. The predominant role of surgery takes place either initially in small localized tumors or later, after initial reductive chemotherapy. In all cases, complete resection is the necessary but not sufficient condition for cure. Additional radiotherapy seems ineffective. High-dose chemotherapy and/or liver transplantation can be proposed for resistant cases. The disease-free survival rate is 37% at 2 years for the whole series.