ABSTRACT
In thinking about cancers that may be amenable to specific therapeutic approaches, one is drawn to the relatively small group of neoplasms, mainly leukemias, lymphomas and sarcomas, that have tumor specific chromosomal rearrangements that encode tumor-specific fusion oncoproteins. Because these are novel proteins, not present in any normal cell, they and the oncogenic pathways they de-regulate may serve as spe cific targets for intervention. An additional fea ture o f these diseases that makes them inter esting objects for research is the high degree o f specificity o f the genetic rearrangement for any given tumor type. It seems likely there fore that such tumors will provide important clues to the understanding o f mechanisms involved in cell-type specificity o f oncogene action. This review focuses on the TLS-CHOP fusion oncogene implicated in human myxoid and round cell liposarcoma. We will attempt to analyze the role o f the encoded oncoprotein in tumor development both from the perspective o f oncogenic transformation in the adipose lin eage and from the broader perspective o f possi ble mechanisms that are shared between all neo plasms that are associated with translocations that lead to fusion o f aTLS-type (or EWS-type) domain to a transcription factor.
