Actively Recruiting
NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
Led by St. Jude Children's Research Hospital · Updated on 2026-05-19
139
Participants Needed
6
Research Sites
156 weeks
Total Duration
On this page
AI-Summary
What this Trial Is About
This research focuses on patients diagnosed with non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), evaluating treatments based on risk categories: low, intermediate, and high risk. The study aims to estimate survival rates, find safe and effective dosages of certain drugs, and explore how the body's processing of medicines relates to treatment effects. It also investigates various outcomes such as tumor control, side effects, genetic tumor features, and cardiovascular health among children and young adults. Participants will receive treatments tailored to their risk and tumor characteristics. Surgery to remove the tumor is standard for all risk groups. Radiation therapy is given to those with positive tumor margins, usually starting 3 to 6 weeks after surgery and lasting 5 to 6 weeks. Intermediate- and high-risk patients may receive chemotherapy drugs including ifosfamide, doxorubicin, pazopanib, and for high-risk patients, selinexor. Maintenance therapy with pazopanib for six months follows consolidation chemotherapy and radiation as appropriate. Some patients may have delayed surgery or additional chemotherapy cycles before surgery. During the study, participants will undergo regular monitoring including evaluations of tumor status, side effects, heart and lung function, and laboratory tests. Researchers will study survival outcomes, treatment side effects, drug levels in the body, and tumor genetic changes using advanced sequencing methods. The study follows participants for multiple years to measure event-free survival and to determine safe dosing levels of selinexor. Close observation continues after treatment to watch for tumor recurrence and long-term health effects.
CONDITIONS
Brief Title
NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Patients must be 30 years old or younger at diagnosis of NRSTS.
- Patients with low-grade tumors of any size with planned or achieved complete or near-complete surgical removal.
- Patients with high-grade tumors smaller than 5 cm with planned or achieved complete or near-complete surgical removal.
- Patients with intermediate-risk disease including unresectable low-grade or high-grade tumors with planned delayed surgery.
- Patients with high-grade tumors larger than 5 cm that may be surgically removable.
- Patients with high-risk disease including metastatic disease, unresectable tumors without planned surgery, or CIC-DUX4 sarcoma.
- Adequate organ function including kidney, liver, heart, and lung function as defined by specific laboratory and clinical criteria.
- Performance status score of 60 or higher on Lansky (for age 16 or younger) or Karnofsky (for older than 16).
- Patients on stable doses of certain blood thinners if treated for at least 6 weeks.
- Life expectancy of at least 3 months with therapy.
- No prior chemotherapy, radiation, or targeted therapy for NRSTS.
- Negative pregnancy test and agreement to use effective contraception if applicable.
You will not qualify if you...
- Patients with primary brain sarcoma or brain metastases.
- Patients with specific sarcoma types such as rhabdomyosarcoma, malignant rhabdoid tumor, or others listed.
- Active bleeding or bleeding disorders, including significant hemoptysis in patients older than 17.
- Uncontrolled high blood pressure.
- Prior systemic therapy for NRSTS including chemotherapy, radiation, or targeted drugs like pazopanib.
- Use of certain medications affecting drug metabolism or heart rhythm within specified timeframes before enrollment.
- Conditions impairing oral drug absorption such as major stomach or bowel surgery, active ulcers, or malabsorption.
- Recent serious blood clots or arterial thrombosis.
- Serious non-healing wounds or ulcers.
- Uncontrolled infections or serious heart or psychiatric conditions limiting study participation.
- HIV-positive patients on combination antiretroviral therapy.
- Use of other investigational agents.
- Pregnancy or breastfeeding, or unwillingness to use contraception during and after the study.
AI-Screening
AI-Powered Screening
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Your Study Journey
Duration - 2 to 4 weeks
Participants are screened for eligibility to participate in the trial.
1 visit (in-person)
Duration - Day 1
Participants will have surgery to remove the tumor as part of standard care. The extent and type of surgery depend on the risk group and tumor characteristics.
1 visit (in-person)
Duration - About 5 to 6 weeks starting 3 to 6 weeks post-surgery
For participants with positive tumor margins, proton beam radiation therapy will start about 3 to 6 weeks after surgery. Radiation is given daily (Monday through Friday) for about 5 to 6 weeks.
Daily visits Monday through Friday for about 5 to 6 weeks
Duration - Up to 3 to 4 cycles over several weeks before or after surgery
Participants at intermediate and high risk receive chemotherapy including ifosfamide, doxorubicin, and selinexor depending on risk group. Chemotherapy cycles occur before or after surgery as part of consolidation therapy.
Multiple visits for chemotherapy infusions over several weeks
Duration - 6 months
Participants in intermediate and high-risk groups receive maintenance therapy with pazopanib by mouth daily for 6 months following consolidation therapy.
Regular visits during 6 months of oral medication
Duration - Up to 3 years or longer
Participants are closely monitored after completion of therapy for tumor recurrence and side effects from treatment.
Periodic follow-up visits for monitoring
Trial Site Locations
Total: 6 locations
1
Lurie Children's Hospital of Chicago
Chicago, Illinois, United States, 60611
Actively Recruiting
2
Our Lady of the Lake Children's Hospital
Baton Rouge, Louisiana, United States, 70809
Actively Recruiting
3
Dana Farber Cancer Institute
Boston, Massachusetts, United States, 02215
Actively Recruiting
4
Washington University Medical Center
St Louis, Missouri, United States, 63110
Actively Recruiting
5
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio, United States, 45229
Actively Recruiting
6
St. Jude Children's Research Hospital
Memphis, Tennessee, United States, 38105
Actively Recruiting
Research Team
J
Jessica Gartrell, MD
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
NON_RANDOMIZED
Model
FACTORIAL
Primary Purpose
TREATMENT
Number of Arms
6
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