Multidisciplinary management of hepatic malignant turners in children. Report of six cases

Background: Malignant hepatic tumors (Mht) are rare in children. Among them hepatoblastoma (HB) is the most common. Aim: To report the results of the multidisciplinary management in 6 consecutive children: five HB and one met astatic Wilms tumor (MWT). Patients and methods: The mean age of patients w as 42 months. All HB patients had elevated serum alfafetoprotein (median 15 0, 000 ng/ml). All patients received preoperative chemotherapy: HB patients received carboplatin/doxorrubicin alternating with cisplatin, and the MWT patient, vincristine alone. Surgery included two formal right and two forma l left hepatectomies, one extensive central resection with partial left seg mentectomy, and one lateral segmentectomy. Extracorporeal circulation was u sed in the child with atrial involvement. All patients received postoperati ve chemotherapy. Results: All tumors had variable regression on preoperativ e chemotherapy. Complete resection with negative margins was achieved in al l patients. The degree of tumor necrosis on histology ranged from 60% to 90 %. Alfafetoprotein levels fell to under 10 ng/ml in all HB cases, one to th ree months after surgery. all patients survive free of disease at a median follow up of 19 months. Conclusion: A multidisciplinary approach including the well timed used of chemotherapy and surgery is highly effective in the management of pediatric malignant tumors.