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Childhood Liver Cancer Treatment (PDQ®)

Health Professional Version

Changes to This Summary (03/05/2015)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information

Added text to state that between 1975 and 2010, childhood cancer mortality decreased by more than 50% (cited Smith et al. as reference 1).

Added text to state that neonates with hepatoblastoma have comparable outcomes to older children up to age 5 years (cited Trobaugh-Lotrario et al. as reference 13). Also revised text to state that the overall 5-year survival rate is 42% for those with hepatocellular carcinoma.

Revised Table 1 to include Aicardi syndrome as a disorder associated with hepatoblastoma (cited Kamien et al. as reference 15).

Added Spector et al. as reference 26.

Cellular Classification

Added text to state that fibrolamellar carcinoma histology is characterized by a fusion transcript created by deletion of a 400 kb section of chromosome 19, which was found in 15 of 15 tumors that were tested (cited Honeyman et al. as reference 13).

Added Weeda et al. reference 18 and level of evidence 3iiA.

Added Olson et al. as reference 23.

Stage Information

Added text to state that revisions and radiologic examples of the PRETEXT staging system were published in 2007 (cited Roebuck et al. as reference 5).

Treatment Option Overview

Revised text to state that the International Society of Pediatric Oncology Epithelial Liver Tumor Group (SIOPEL) has reported a pilot study of high-risk hepatoblastoma patients; in SIOPEL-3HR, cisplatin alternating with carboplatin/doxorubicin was administered in a dose intensive fashion. Also added text to state that in a second trial, cisplatin was dose-intensified in a single-arm prospective study; 3-year event-free survival (EFS) was 76% and overall survival (OS) was 83%; toxicity was significant but acceptable (cited 2013 Zsiros et al. as reference 28 and level of evidence 2A).

Added Antiviral Treatment of Hepatitis B Virus (HBV)–related Hepatocellular Carcinoma as a new subsection.

Added text to state that chemotherapy followed by transarterial chemoembolization (TACE) followed by high-intensity focused ultrasound showed promising results in China for PRETEXT III and IV patients, some of whom were resectable but did not undergo surgery because of parent refusal (cited Wang et al. as reference 40).

Added text to state that transarterial radioembolization with Yttrium-90 resin beads has been used to palliate children with hepatocellular carcinoma (cited Hawkins et al. as reference 47).

Treatment of Hepatoblastoma

Added text about treatment options for stage IV to state that a prospective feasibility trial of dose-dense, cisplatin-based chemotherapy and radical surgery in 62 patients with high-risk hepatoblastoma resulted in a 3-year EFS of 76% and 3-year OS of 83%. In this study, of 37 patients with distant metastases, 27 were surviving disease free at 3 years (cited 2013 Zsiros et al. as reference 22 and level of evidence 3iiDi).

Added 2012 Zsiros as reference 26 and level of evidence 3iiA.

Treatment of Undifferentiated Embryonal Sarcoma of the Liver

Added Plant et al. as reference 9 and level of evidence 3iiiA.

Treatment of Recurrent Childhood Liver Cancer

Revised text to state that a phase II Children's Oncology Group trial of single-agent sorafenib has been completed in children and the study results are pending.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.

  • Updated: March 5, 2015