Sarcomas are malignant tumors that affect connective tissues. While generally rare, in patients with DICER1 syndrome, sarcomas are not rare. They primarily arise in the brain and the urogenital tract (e.g. cervix) at a young age, leading to potentially severe consequences. Existing treatments, namely surgery and systemic chemotherapy, do not often lead to positive outcomes. This is due to the inaccessibility of many of the tumors, their inherent chemoresistance and long-term secondary health issues that result from intervention, such as infertility.

Research Institute of the McGill University Health Centre
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