Genome-Wide Profiles of Extra-cranial Malignant Rhabdoid Tumors Reveal Heterogeneity and Dysregulated Developmental Pathways

Genome-Wide Profiles of Extra-cranial Malignant Rhabdoid Tumors Reveal Heterogeneity and Dysregulated Developmental Pathways
Chun, HJE, (2016) Cancer Cell (Link to abstract)

 

This study analyzes genomes, transcriptomes, DNA methylation, histone signatures, and microRNA in extracranial rhabdoid tumors. There are many different findings reported in this paper but in their totality they are evidence that rhabdoid tumors have heterogeneous molecular features despite all being initially driven by SMARCB1 mutations. Different techniques reveal slightly different subgroups but it is likely that there are two major groups of rhabdoid tumors – each seems to arise from a different neural crest cell precursor.


* When compared with similar studies in AT/RT – a very similar tumor, this study doesn’t appear to reveal as many clinically-relevant findings. However, this is the first comprehensive analysis of rhabdoid tumor molecular features and is therefore an important starting point for treating these very difficult tumors.