Title:Hepatoblastoma: Review of Pathology, Diagnosis and Modern Treatment Strategies
VOLUME: 16 ISSUE: 4
Author(s):Adil A. Abbas*, Alaa M.N. Samkari and Abeer S. Almehdar
Affiliation:Pediatric Hematology/Oncology Section, College of Medicine, Princess Nourah Oncology Centre, King Saud Bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Jeddah, Department of Laboratory Medicine, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Jeddah, Department of Medical Imaging, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Jeddah
Keywords:Alfa fetoprotein, β-catenin, cisplatin, hepatoblastoma, liver transplantation, pretext.
Abstract:Hepatoblastoma (HB) is the most common primary malignant hepatic tumor of childhood
and, occurring predominantly in the first two years of life. Approximately 100 cases are diagnosed
every year in the United States of America. The management of HB has changed markedly
over the last three decades. Alfa feto protein (AFP) and beta human chorionic gonadotrophin (beta
HCG) are the main tumor markers and are markers for diagnosis and follow up. International collaborative
efforts have led to the implementation of the Pre - Treatment Extent of the Disease PRETEXT
staging system consensus classification to assess upfront resectability. Complete surgical
resection plays a key role in successful management. Overall, outcomes have greatly improved over
the past decades mainly because of advances in chemotherapy (CTR) agents and administration
protocols, newer surgical approaches and liver transplantation (LT). Targeted medications towards
the newly discovered β-catenin and Wnt genetic pathways in tumor cells may soon become an option
for treatment. The current disease free survival (DFS) rates are approaching 85%. For the 25%
of patients with metastasis at presentation, the overall survival (OS) remains poor. A more individualized
approach to treating the heterogeneous spectrum of HB may become the basis of successful
treatment in complex cases. Newer medications and surgical techniques are being exploited.
Here we present a comprehensive review of the recent advances in the management of HB. A wide
literature search was made using internet search engines such as PubMed and Google scholar. More
than 100 articles were reviewed and the information extrapolated was arranged to produce this
review.
