Patients participating in this study have DSRCT that has spread locally or to other parts of the body and can no longer be surgically removed without causing significant harm.
Treatment will continue until the tumor progresses further, severe side effects occur, or either patient or investigator decision.
In addition, patients may participate in an optional biological study. The study will analyze the tumor's genes and the molecules related to them. By studying genes and their products, the investigators can better understand the behavior of the tumor and how the body responds to therapies.
Histological centrally confirmed diagnosis of DSRCT with the documented presence of EWSR1-WT1 translocation.
Age ≥ 15 years.
Locally advanced (i.e. radical surgical resection of local disease unfeasible or surgery declined by the patient or surgery deemed to become less demolitive and / or easier after cytoreduction) and/or metastatic disease.
Measurable disease by RECIST v1.1.
Prior treatment with lurbinectedin or trabectedin, Ecubectedin (PM 14) or PM54.
Known hypersensitivity to irinotecan or lurbinectedin or any of their components of the drugs products (excipients).
Other primary malignancy with <5 years clinically assessed disease free interval, except basal cell skin cancer, cervical carcinoma in situ or other neoplasm judged to entail a low risk of relapse.
History or presence of unstable angina, myocardial infarction, or clinically significant valvular heart disease within 12 months of the study.