Discovery of the origin of malignant rhabdoid tumor in childhood cancer

Discovery of the origin of malignant rhabdoid tumor in childhood cancer

The first proof of the origin of the malignant rhabdoid tumor (MRT), a rare childhood cancer, has been discovered by researchers at the Wellcome Sanger Institute, the Princess Máxima Center for Pediatric Oncology in the Netherlands and their collaborators.

The study, published today (March 3, 2021) in Nature Communications, found that MRT originates from developmental neural crest cells * whose maturation is blocked by a genetic defect. The team also identified two drugs that could be used to overcome this blockage and resume normal development, offering hope for new treatments for the disease.

Malignant rhabdoid tumor (MRT) is a rare soft tissue cancer that primarily affects infants. Although these tumors can occur in any part of the body, they usually form in the kidneys and brain. MRT is one of the poorest childhood cancers.

The rarity of MRT, with only 4-5 cases per year in the UK, combined with its aggressiveness, makes clinical trials extremely difficult. Until now, the origin of MRT was not known and there is currently no reliable and effective treatment. This new study sought to uncover the root of MRT in the hope of identifying new treatments …

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