Mechanism of action of trabectedin in desmoplastic small round cell tumor cells.

Sarah Uboldi, I. Craparotta, G. Colella, E. Ronchetti, L. Beltrame, S. Vicario, S. Marchini, Nicolò Panini, G. Dagrada, F. Bozzi, S. Pilotti, C. M. Galmarini, M. D'Incalci, R. Gatta

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive disease, that can be described as a member of the family of small round blue cell tumors. The molecular diagnostic marker is the t(11;22)(p13;q12) translocation, which creates an aberrant transcription factor, EWS-WT1, that underlies the oncogenesis of DSRCT. Current treatments are not very effective so new active drugs are needed. Trabectedin, now used as a single agent for the treatment of soft tissue sarcoma, was reported to be active in some pre-treated DSRCT patients. Using JN-DSRCT-1, a cell line derived from DSRCT expressing the
Original languageUndefined/Unknown
Pages (from-to)107
Number of pages1
JournalBMC Cancer
Issue number1
Publication statusPublished - Feb 1 2017


  • DSRCT, JN-DSRCT-1, Trabectedin

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