Pediatric liver tumors and hepatic ontogenesis: common and distinctive pathways.

Abstract

Several types of pediatric liver tumors exhibit structural features apparently reflecting processes which normally occur during hepatic ontogenesis: some hepatoblastomas mimic distinct phases of hepatogenesis, including the formation of mesenchymal structures closely associated with immature epithelia, and there are tumors almost exclusively consisting of complex mesenchymal patterns. Current classifications of hepatoblastomas refer to the identification of more or less mature (differentiated) single or mixed components seen in histologic preparations. These do not, however, attempt to integrate ontogenic pathways, in contrast for example, to nephroblastoma and associated lesions, where such a view has proved to be highly fruitful. Based on the fact that an enormous amount of knowledge has recently been accumulated regarding hepatic ontogenesis, time may have come to look at these tumors with a new eye. In what follows, we aim at trying to analyze distinct features of pediatric hepatic tumors (except vascular tumors) within the background of ontogenesis. Some key steps of hepatogenesis and the regulatory factors involved may, in the future, deliver an armamentarium to search for novel molecular mechanisms involved in tumorigenic pathways.