Long-term survival in children with primary hepatic malignancies can not be expected without complete tumor resection. In the last ten years we have treated 21 children with hepatocellular carcinoma (HCC) and 21 children with hepatoblastoma (HEP), with tumor extirpation our surgical goal. Operative treatment included partial hepatectomy ([PH] 20), either primary (10) or delayed (following chemotherapy) (10), total hepatectomy and orthotopic liver transplantation ([OLT] 13), or upper abdominal exenteration and multiple organ transplantation (2). Two patients had both PH and subsequent total hepatectomy and OLT. Overall survival was 48% (20/42), with 9 patients dying of progressive disease prior to removal of their tumor. HEP patient survival was 67% (14/21), including 2 of 6 who underwent primary PH, 7 of 8 who had delayed PH, and 5 of 6 who underwent OLT. Survival for the children with HCC was 29% (6/21), including 1 of 4 after primary PH, 1 of 2 following delayed PH, 3 of 7 following OLT, and 1 of 2 after exenteration and multiple organ transplantation. Preoperative chemotherapy facilitated removal of 10 initially unresectable tumors (8 HEP, 2 HCC) at a second-look procedure. Total hepatectomy and OLT markedly improved survival in patients with disease unresectable by standard methods. Partial hepatectomy, either primary or delayed, should be attempted in all children with hepatic malignancies. Total hepatectomy and OLT appears to be a viable adjunct in the treatment of childhood malignancies, and should be used for otherwise unresectable tumors as part of a carefully planned protocol.
INDEX WORDSHepatoblastoma; hepatocellular carcinoma; liver transplantation Childhood liver tumors continue to have a poor prognosis despite increasingly sophisticated diagnostic methods, aggressive surgical techniques to achieve complete resection, and more effective systemic chemotherapy. Total surgical removal remains the mainstay of treatment, although there are rare reports of long-term survivors achieved with chemotherapy alone. Preoperative chemotherapy has increased the number of patients undergoing potentially curable resections. 1 However, a large proportion of children, especially with hepatocellular carcinoma (HCC), are not resectable by standard methods. Total hepatectomy and orthotopic liver transplantation (OLT), the only alternative for unresectable tumors, is still controversial due to the high incidence of tumor recurrence. 2Address reprint requests to Edward P. Tagge, MD,
