Hepatoblastoma state of the art: pre-treatment extent of... : Current Opinion in Pediatrics (original) (raw)

HEMATOLOGY AND ONCOLOGY: Edited by Stephen P. Hunger

pre-treatment extent of disease, surgical resection guidelines and the role of liver transplantation

Meyers, Rebecka L.a; Tiao, Gregb; de Ville de Goyet, Jeanc; Superina, Riccardod; Aronson, Daniel C.e

aPediatric Surgery, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah

bPediatric Surgery, Cincinnati Children's Hospital and Medical Center, Cincinnati, Ohio, USA

cDepartment of Pediatric Surgery and Transplantation, Ospedale Pediatrico Bambino Gesù, University of Rome ‘Tor Vergata’, Italy

dPediatric Surgery, Lurie Children's Hospital of Chicago, Chicago, Illinois, USA

eQueen Elisabeth Central Hospital, College of Medicine, University of Malawi, Blantyre, Malawi

Correspondence to Rebecka L. Meyers, MD, Professor of Pediatric Surgery, University of Utah, Pediatric Surgery Suite 2600, Primary Children's Medical Center, 100 Mario Capecchi Dr., Salt Lake City, UT 84113, USA. Tel: +1 801 662 2950; e-mail: [email protected]

Abstract

Purpose of review

This is part two of a two-part state of the art – hepatoblastoma. International hepatoblastoma specialists were brought together to highlight advances, controversies, and future challenges in the treatment of this rare pediatric tumor.

Recent findings

Pretreatment extent of disease (PRETEXT) is a grouping system introduced as part of the multicenter international childhood liver tumors strategy group, SIOPEL-1, study in 1990. The system has been refined over the ensuing years and has now come to be adopted for risk stratification by all of the major pediatric liver tumor multicenter trial groups. PRETEXT is being intensively studied in the current Children's Oncology Group (COG) AHEP-0731 trial in an attempt to validate interobserver reproducibility and ability to monitor response to neoadjuvant chemotherapy, and determine surgical resectability. PRETEXT is now used to identify those patients who are at risk for having an unresectable tumor and who should be referred to a liver specialty center with transplant capability early in their treatment schema.

Summary

International collaborative efforts in hepatoblastoma have led to increased refinements in the use of the PRETEXT and post-treatment extent to define prognosis and surgical resectability. PRETEXT criteria which suggest a possible need for liver transplantation are discussed in detail.