Surgical management of children with locally advanced hepatoblastoma


We read with great interest the article by Lautz et al titled “Successful nontransplant resection of POST-TEXT III and IV hepatoblastoma.”1 The authors document excellent results with a difficult subgroup of children with hepatoblastoma. We agree with their conclusion that referral to a center specializing in pediatric hepatobiliary surgery should not be considered a referral for a predestined liver transplant, even if the child has a hepatoblastoma that is extensive and potentially unresectable. Rather, it is a referral to a center where the expertise is available to perform a complex liver resection or a transplant, in children, where it is often not possible to know which is the better treatment in a disease where complete surgical resection is essential for cure.

This article points out the challenges of providing definitive surgical care for such children. One child died and 2 of the 13 survivors had major vascular complications: 1 required urgent salvage liver transplant for ischemic cholangiopathy and 1 required a splenorenal shunt for postoperative thrombosis of the portal vein. Even in experienced hands, such major vascular complications will occur. This risk, plus the extensive literature showing improved oncology outcomes with upfront, rather than “rescue,” liver transplant, has driven the increased and highly successful use of liver transplantation in this disease over the past decade.

We question the authors' conclusion that PRETEXT should not be used as a triage tool because it has a tendency to overstage. The PRETEXT system has been used effectively in European SIOPEL [Liver tumor study group of the International Society of Pediatric Oncology (SICP)] multicenter studies to triage those children with aggressive tumors to the centers best able to manage the operative challenges the authors note. SIOPEL multicenter studies 1, 2, and 3 have documented improved patient survival with this approach. In fact, one of the major study objectives of AHEP-0731 is to determine the accuracy of the PRETEXT/POST-TEXT group in predicting surgical resection.

Finally, and perhaps most importantly, we would call attention to a small but very important misstatement in the article's quotation of the surgical guidelines in the current Children's Oncology Group (COG) protocol AHEP-0731 “Treatment of Children with all Stages of Hepatoblastoma.” In the last sentence on page 1976, the authors state, “Children with POST-TEXT III and IV tumors are referred to a surgical center with expertise in pediatric liver transplant and extreme liver resection at diagnosis if possible and no later than just after the second cycle of chemotherapy.” First, the correct terminology for the chemotherapy classification at diagnosis is PRETEXT; classification after neoadjuvant chemotherapy is POST-TEXT. Second and more importantly is to emphasize that COG AHEP-0731 does not recommend referral of all patients with PRETEXT/POST-TEXT III tumors, only the subset of children with POST-TEXT III tumors who demonstrate persistent major vascular involvement after neoadjuvant chemotherapy. POST-TEXT III tumors without major vascular involvement are considered “resectable” by the COG AHEP-0731 protocol and do not need to be referred to a specialty liver center. AHEP-0731 does recommend referral for all patients with POST-TEXT IV tumors. Because of inferior outcomes seen in previous large multicenter studies, the protocol does not recommend piecemeal nonanatomic resection of these POST-TEXT IV tumors when they are multifocal.

Under ideal circumstances, we would suggest that all children in the United States with hepatoblastoma should be enrolled in AHEP-0731 and wish to assure readers that the COG surgical guidelines are just that: they are guidelines. Our committee would welcome the opportunity to clarify any questions surgeons or oncologists might have regarding this study, which was designed with the goal espoused in the article's conclusion “…to provide the best oncologic therapy for each individual child while limiting unnecessary long-term morbidity.”

Rebecka L. Meyers MD*, Gregory M. Tiao MD†, Stephen P. Dunn MD‡, Eugene D. McGahren III MD§, Max R. Langham Jr MD¶, * University of Utah, Primary Children's Medical Center, Salt Lake City, Utah, † Cincinnati Children's Hospital and Medical Center, Cincinnati, Ohio, ‡ Alfred I. duPont Hospital for Children, Wilmington, Delaware, § University of Virginia, Charlottesville, Virginia, ¶ University of Tennessee, Le Bonheur Children's Hospital, Memphis, Tennessee.