NCT07328425

Clinical Study in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT) ISG-TULIPS

Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT)

• Status: Recruiting
• Phase: Phase 2
• Study type: Interventional
• Enrollment: 20 (estimated)
• Sponsor: Italian Sarcoma Group · Network
• Sex: All
• Age: 15 Years
• Healthy volunteers: Not accepted

Summary

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.

Detailed description

This is an Italian and Spanish multicentric, prospective, phase II single-arm, open-label, investigator-initiated clinical study that will be conducted within the Italian Sarcoma Group (ISG) and the Spanish Sarcoma Groups (GEIS), aiming at exploring the activity of lurbinectedinin combination with irinotecan, until progression or unacceptable toxicity, in a population of =/\>15 years old patients with histologically and molecularly confirmed (EWSR1-WT1 translocation positive), advanced (locally advanced or metastatic) DSRCT, from 2nd to 4th line, following progression to anthracycline-based chemotherapy. The primary end-point of the study will be the overall response rate (ORR) by RECIST v1.1 in the study population. Secondary end-points will be PFS, DoR, OS, safety and changes in QoL. Patients will be evaluated for the primary end-point if have completed at least one cycle of lurbinectedin and irinotecan and have at least one post-baseline radiologic disease assessment. Patients with documented early progression disease (PD) or who die due to PD before the first scheduled tumor assessment will also be included in the analysis. Patients will be followed for follow up visits every 6 months for 2 years.

Conditions

• Desmoplastic Small Round Cell Tumor

Interventions

Type	Name	Description
DRUG	Lurbinectedin	Lurbinectedin and Irinotecan
DRUG	Irinotecan	Lurbinectedin and Irinotecan

Timeline

• Start date: 2025-11-21
• Primary completion: 2030-10-01
• Completion: 2030-10-01
• First posted: 2026-01-09
• Last updated: 2026-04-03

Locations

1 site across 1 country: Italy