Where do we stand with hepatoblastoma? A review.
Hepatoblastoma (HB) is the most common pediatric liver malignancy, comprising approximately 1% of all pediatric cancers. The disparate clinical staging systems and histologic classifications that were developed during the last decades, nevertheless, reflect the remaining difficulties and uncertainties in characterizing HB. Furthermore, the combination of surgery and (neo)adjuvant chemotherapy has improved patient outcomes dramatically. A poor prognosis is associated with large tumor size, multifocality, extrahepatic disease, and metastatic spread. The exact etiology of HB remains unknown, but the cytogenetic alterations, phenotypic features, and biologic aspects that accompany this neoplasm yield more and more insight into its pathogenesis. New cell-biologic and molecular-biologic insights may lead to the development of new treatment modalities, especially for patients with a bad prognosis. This review summarizes the different aspects of this intriguing tumor and discusses the current status of research and treatment for patients with HB.
Schnater
J Marco
JM
Pediatric Surgical Center of Amsterdam, Emma Children's Hospital AMC, Academic Medical Center, Amsterdam, The Netherlands.
Köhler
S Eleonore
SE
Lamers
Wouter H
WH
von Schweinitz
Dietrich
D
Aronson
Daniël C
DC
D002648:Child; D018197:Hepatoblastoma / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D009367:Neoplasm Staging; D011379:Prognosis