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NRSTS2021: A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy, and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma

About this study

Non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) consists of a group of tumors (cancers) that occur in the soft tissues of the body, such as the muscles. The treatment for this cancer is based on how abnormal the tumor cells look (tumor grade), how big the tumor is, if it can be surgically removed, and if it has spread to other parts of the body (metastasized).

Using this information, NRSTS patients are usually classified into 3 groups. These groups are based on the patient’s risk of cancer returning after treatment. These groups are low-, intermediate-, and high-risk. While most NRSTS in low-risk patients can be cured with surgery with or without radiation therapy, better treatments with fewer side effects are needed for those in the intermediate- and high-risk groups.

Purpose of this clinical trial

In this clinical trial, we hope to find better treatments for NRSTS patients who are intermediate- and high-risk. Our goal is to lower the risk of the tumor coming back after treatment, while using treatments with fewer risks of long-term side effects.

Patients in this study will receive treatment based on their risk:

  • Low-risk patients will be treated with surgery to remove the tumor and, if needed, radiation therapy (protons or X-rays) to kill cancer cells.
  • Intermediate-risk patients will receive standard chemotherapy with anti-cancer drugs, surgery, and radiation therapy (protons or X-rays). As part of their treatment, these patients will also receive a new chemotherapy drug called pazopanib. This drug was approved by the United States Food and Drug Administration (FDA) for adult patients with other cancer and was recently shown to be safely tolerated in children and young adults with NRSTS.
  • High-risk patients will receive standard chemotherapy with anti-cancer drugs, surgery, and radiation therapy (protons or X-rays). As part of their treatment, these patients will also receive pazopanib and a new chemotherapy drug, selinexor. We want to find the safest dose of selinexor to use and better treat children with high-risk NRSTS.

Eligibility overview

To take part in this study, you must be:

  • Newly diagnosed non-rhabdomyosarcoma soft tissue sarcoma
  • Up to 30 years old

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Overview

Full title:

A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy, and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma

Study goal:

The purpose of this clinical trial is to find better treatments for NRSTS patients who are intermediate- and high-risk. Our goal is to lower the risk of the tumor coming back after treatment, while using treatments with fewer risks of long-term side effects.

Diagnosis:

Non-rhabdomyosarcoma soft tissue sarcoma, adipocytic neoplasm, liposarcoma, synovial sarcoma, MPNST, undifferentiated sarcoma

Age:

Up to 30 years old.

Clinical trials categories:

Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS) Childhood Cancer Epithelioid Hemangioendothelioma Synovial Sarcoma

For physicians and researchers

Patients accepted to St. Jude must be referred by a physician or other qualified medical professional. Learn how St. Jude can partner with you to care for your patient.

 

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