ERC awards €1.5 million to research into rhabdoid tumors

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Credit: Princess Máxima Center

Rhabdoid tumors are aggressive tumors that occur in the brain, kidneys and soft tissues. Every year five to ten children in the Netherlands are diagnosed with a rhabdoid tumor, generally at very early age. In Europe these are approximately a thousand children. The cells of a rhabdoid tumor divide very rapidly and often the disease already metastasized at time of diagnosis. Chemotherapy seldom cures the children, and prognosis is therefore very dismal. ‘The disease is rare, but most children with a rhabdoid tumor die within a few months after diagnosis’, says Drost.

Tumor tissue

Since 2016, Drost leads his own research group in the Princess Máxima Center and specializes in research into rhabdoid tumors. Since the opening of the center, nearly all children with cancer in the Netherlands are treated in the Máxima. This entails that doctors and researcher in Utrecht see all patients with a rhabdoid tumor. ‘This allows us to collect material for research from relatively many different patients, despite the rareness of the tumor type’, explains Drost. ‘That is essential to study specific characteristic of the tumor cells in detail.’

Characteristics of the tumor

One of the features is that rhabdoid tumors often develop resistance in a short period of time to the given chemotherapy. Drost hypothesizes two possible explanations: ‘Either the tumor cells adapt very quickly to the treatment and become resistant to it, or resistant cells are already present even before the treatment starts. These cells survive treatment and repopulate the tumor. It is crucial to know which of the two explanations is true in order to treat the cancer effectively.’

With the ERC Starting grant, Drost will focus on the heterogeneity of primary rhabdoid tumors, charateristics of the metastases and the developmental origin of the tumor cells. By means of unique modelsystems (organoids) and primary tumor tissue in combination with innovative lineage tracing techniques, Drost will examine which features are underlying the aggressive nature of rhabdoid tumors.

Prestigious starting grant

The ERC recognizes the importance and the innovative character of Drost’s research. With the amount of 1.5 million euro, Drost can expand his research group in the next five years. In total 3108 scientist applied for the ERC starting grant, which was ultimately awarded to 13 percent of the applicants.

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