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Preliminary Clinical Study of Entinostat in Children With Malignant Soft Tissue Sarcoma Harboring PAX-family Gene Abnormalities
Sponsor: Beijing Tongren Hospital
Summary
Pediatric soft tissue sarcomas (STS) are a group of malignant tumors originating from primitive mesenchymal tissues and account for approximately 7% of childhood cancers. STS are broadly classified into rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS). RMS accounts for approximately one half of pediatric STS. As reported, PAX fusion-positive RMS is more aggressive than fusion-negative RMS, with higher metastatic potential and poorer prognosis. Based on the unmet therapeutic need in pediatric sarcoma with PAX-family abnormalities, this study is intended to evaluate the preliminary safety and antitumor activity of entinostat with or without chemontherapy.
Official title: A Single-Center Clinical Study Evaluating the Safety and Antitumor Activity of Entinostat in Children With Malignant Soft Tissue Sarcoma Harboring PAX-family Gene Abnormalities
Key Details
Gender
All
Age Range
1 Year - 18 Years
Study Type
INTERVENTIONAL
Enrollment
16
Start Date
2025-03-15
Completion Date
2027-03-31
Last Updated
2026-10-22
Healthy Volunteers
No
Conditions
Interventions
Entinostat
For patients aged 1-3 years or weighing \<40 kg: starting dose 3 mg orally once weekly, maintained at 3 mg. For patients aged \>3 years or weighing ≥40 kg: starting dose 3 mg orally once weekly, with gradual escalation to a maintenance dose of 5 mg, or a starting dose of 5 mg.
VAC/VTC chemotherapy
VAC regimen: vincristine 1.5 mg/m² on Day 1; dactinomycin 12-15 µg/kg on Days 1-5; and cyclophosphamide 300 mg/m² on Days 1-4. VTC regimen: vincristine 1.5 mg/m² on Day 1; topotecan 1.2 mg/m² on Days 1-5; and cyclophosphamide 250 mg/m² on Days 1-5. If prior treatment with the VAC/VTC regimen is ineffective because of resistance or is not tolerated for safety reasons, the investigator team will discuss and develop an individualized combination chemotherapy regimen
Locations (1)
Beijing Tongren Hospital
Beijing, Beijing Municipality, China