期刊
HUMAN PATHOLOGY
卷 40, 期 6, 页码 843-853出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.humpath.2008.10.022
关键词
Gene expression profiling; Fetal; Small cell and embryonal hepatoblastoma; Liver tumors; Pediatric
类别
资金
- NCI NIH HHS [U10 CA098543, U10 CA098543-07, U10 CA098413-05, U10 CA098543-01, U10 CA098413-04] Funding Source: Medline
Hepatoblastoma is the most common malignant turner of the liver of children worldwide. Histologically, hepatoblastomas show marked variation in the type and proportion of epithelial (fetal, embryonal, or small cell) and mesenchymal components with differing prognosis and response to therapy. The pure fetal-type hepatoblastoma, presenting as stage 1 and resectable, has the best prognosis, whereas the small cell histology has been associated with unfavorable outcome. Using gene expression profiling, we demonstrate that in addition to Wnt pathway deregulation, cell growth and survival pathways are also globally deregulated in hepatoblastomas. Furthermore, the different histologic subtypes are characterized by specific gene expression and pathway signatures that give insight into the degree of molecular heterogeneity that is present among these turners. Although Wnt signaling pathway upregulation is common to all histologic types of hepatoblastoma, this pathway is even more significantly deregulated in aggressive hepatoblastomas. In addition, deregulation of MAPK signaling pathway and antiapoptotic signaling is preferentially upregulated in aggressive epithelial hepatoblastomas with a small cell component. The gene expression signatures reported here provide possible prognostic and diagnostic markers as well as therapeutic targets for this disease. (C) 2009 Elsevier Inc. All rights reserved.
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