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World J Transplant. Dec 24, 2014; 4(4): 294-298
Published online Dec 24, 2014. doi: 10.5500/wjt.v4.i4.294
Role of liver transplantation in the management of hepatoblastoma in the pediatric population
Saira Khaderi, Jacfranz Guiteau, Ronald T Cotton, Christine O’Mahony, Abbas Rana, John A Goss
Saira Khaderi, Jacfranz Guiteau, Ronald T Cotton, Christine O’Mahony, Abbas Rana, John A Goss, Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX 77030, United States
Author contributions: Khaderi S and Guiteau J wrote the manuscript; Cotton RT and Rana A performed the statistical analysis and assisting in writing the manuscript; O’Mahony C and Goss JA provided expertise regarding pediatric transplantation and assisted in writing the manuscript.
Correspondence to: Saira Khaderi, MD, MPH, Assistant Professor of Surgery, Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, 6620 Main Street, Suite 1425, Houston, TX 77030, United States. khaderi@bcm.edu
Telephone: +1-832-3551400 Fax: +1-713-6102479
Received: August 12, 2014
Revised: October 21, 2014
Accepted: October 28, 2014
Published online: December 24, 2014
Processing time: 139 Days and 8 Hours
Abstract

Hepatoblastoma (HB) is the most common primary liver tumor in children and accounts for two-thirds of all malignant liver neoplasms in the pediatric population. For patients with advanced HB (unresectable or unresponsive to chemotherapy), combined treatment with chemotherapy and liver transplantation is an excellent option. The etiology of HB is mostly obscure because of its extreme rarity although some inherited syndromes and very low birth weight have been associated with it. The prognosis for children with HB has significantly improved in the past three decades thanks to advancements in chemotherapy, surgical resection and postoperative care. In 2002 a surgical staging system called pretreatment extent of disease (PRETEXT) was designed to allow a universal, multidisciplinary approach to patients with HB. Between one-third to two-thirds of patients initially present with unresectable tumors or distant metastases, but up to 85% of these tumors become operable after neoadjuvant chemotherapy. Patients with PRETEXT categories 1, 2, and some 3 are referred for neoadjuvant chemotherapy followed by surgical resection with the goal of complete tumor removal. Classic treatments regimens include a combination of cisplatin, fluorouracil, and vincristine or cisplatin and doxorubicin. Liver transplantation is the only treatment option for unresectable HB. In 2010 the pediatric end-stage liver disease, a pediatric-specific scoring system that determines a patient’s ranking on the liver transplant list, began to award additional “exception” points for patients with HB. We analyzed the Standard Transplant Analysis and Research dataset to assess the impact of changes in exception point criteria for HB on outcomes after liver transplantation at Texas Children’s Hospital in Houston, Texas. We found that patients who were listed for transplantation with current HB exception criteria experienced a shorter waitlist time but survival was similar between the two eras.

Keywords: Liver transplant; Hepatoblastoma; Pediatric; Chemotherapy; Cancer

Core tip: Hepatoblastoma (HB) is the most common primary liver tumor in children. Between one-third to two-thirds of patients present with unresectable tumors or distant metastases, but up to 85% of these tumors become operable after neoadjuvant chemotherapy. Liver transplantation is the only treatment option for unresectable HB. In 2010 the pediatric end-stage liver disease scoring system began to award additional “exception” points for patients with HB. We analyzed the Standard Transplant Analysis and Research dataset and found that patients who were listed for transplantation with current HB exception criteria experienced a shorter waitlist time but survival was similar between the two eras.