J Pediatr Surg 1992 Mar;27(3):292-6; discussion 297

Resection, including transplantation, for hepatoblastoma and hepatocellular
carcinoma: impact on survival

Tagge EP, Tagge DU, Reyes J, Tzakis A, Iwatsuki S, Starzl TE, Wiener ES

Department of Surgery, University of Pittsburgh School of Medicine, PA

Abstract
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