In the Western world, cancer is still the main cause of disease-related death among children. Many childhood solid tumors already originate in the developing fetus. They arise as a result of a block in processes driving lineage specification and differentiation during embryonic development. In most cases, the cells from which the tumors originate are only present during short, specific time windows. As a consequence, it is challenging to identify the processes initiating and driving the tumor. The Drost group studies childhood kidney and rhabdoid tumors and aims to understand their molecular and cellular identity.
For our research, we use primary patient material and stem cell culture models, such as patient-derived cancer organoids, in combination with (single-cell) sequencing and gene editing technologies. We are particularly interested in the (epi)genetic mechanisms driving renal and rhabdoid tumorigenesis. For instance, rhabdoid tumors are genetically “cold” tumors with only one recurrent driver mutation. Still, it is one of the most aggressive childhood cancers, which is most likely caused by epigenetic dysregulation. By understanding how these tumors develop during embryonic development and by performing high-throughput compound screens on patient-derived tumor organoids, we aim to find therapeutic opportunities to treat children with these currently untreatable tumors.