Emanuela Palmerini, MD, Istituto Ortopedico Rizzoli, Bologna, Italy, introduces capicua transcriptional repressor (CIC)-rearranged sarcoma, a type of undifferentiated round cell sarcoma that, until recent advances in sequencing, was undetected and then poorly defined. It is a rare, extremely aggressive tumor type, with the median age of diagnosis around 32 years. 40% of patients present with metastases upon diagnosis, and the 3-year survival rate for such cases is 20%. In the search for more effective treatments, patient-derived tumor xenografts have been established for the capicua-double homeobox 4 (CIC-DUX4)-rearranged sarcoma subtype. These models have responded well to the combination of trabectedin and a dual PI3K/mTOR inhibitor. Further research into the employment of inhibitors is ongoing. This interview took place at the ESMO Sarcoma and Rare Cancers Congress 2023 in Lugano, Switzerland.
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