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Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS) Treatment

A non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) is a tumor that can develop in the body’s soft tissues. There are more than 50 types of NRSTS tumors. They are grouped together because they are rare and usually treated in a similar way.

NRSTS tumors commonly occur in the arms and legs. But they can occur anywhere in the body.

The subtypes of NRSTS together make up 4% of all childhood cancers.

Find out more about NRSTS on the Together by St. Jude™ online resource.

Types of NRSTS tumors

The most common types of NRSTS tumors in children include:

  • Synovial sarcoma: Affects the muscles and tendons, especially near joints. Synovial sarcomas can also form in the lungs and abdomen (belly), but this is rare.
  • Malignant peripheral nerve sheath tumor (MPNST): Affects nerve sheaths (coverings)
  • Undifferentiated sarcoma: Occurs in the soft tissues of the body, typically in the arms or legs, but can happen anywhere.

Other types of NRSTS tumors are:

  • Alveolar soft part sarcoma (ASPS)
  • BCOR-rearranged sarcoma
  • Chondrosarcoma
  • CIC-DUX4 rearranged sarcoma
  • Clear cell sarcoma
  • Dermatofibrosarcoma
  • Desmoid fibromatosis
  • Epithelioid sarcoma
  • Fibrosarcoma
  • Hemangiopericytoma
  • Leiomyosarcoma
  • Liposarcoma
  • Malignant fibrous histiocytoma
  • Non-synovial cell sarcoma
  • Malignant rhabdoid tumor

Treatment of non-rhabdomyosarcoma soft tissue sarcoma

Surgery is the most effective NRSTS treatment. Surgery is used to remove as much of the tumor as possible.

Radiation therapy may also be used with patients who have tumor remaining after surgery or for patients with more aggressive tumor types.

Chemotherapy may benefit certain patients with intermediate and high-risk NRSTS tumors.

Scientists are also studying targeted therapies to treat NRSTS.
 

Non-rhabdomyosarcoma soft tissue sarcoma clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults with NRSTS. Learn more about clinical trials at St. Jude.

Recruiting
NRSTS2021: 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.

Age:

Up to 30 years old.

Non-rhabdomyosarcoma soft tissue sarcoma care at St. Jude

St. Jude provides the highest level of care for patients with NRSTS:

  • Current treatment guidelines for these tumors were explored in a clinical trial that was developed at St. Jude.
  • St. Jude offers a dedicated team of specialists to meet the needs of children with brain tumors, including:

More reasons to choose St. Jude for care include:

  • We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report.
  • At St. Jude, we have created an environment where children can be children and families can be together.
  • We lead more clinical trials for childhood cancer than any other hospital in the U.S. 
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer.
  • The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
  • Patients may be able to get expert, compassionate care and treatment closer to their homes through our St. Jude Affiliate clinics.
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Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact us

Contact the Physician / Patient Referral Office

Submit online referral form

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Fax: 901-595-4011 | Email: referralinfo@stjude.org | 24-hour pager: 1-800-349-4334

The solid tumor coordinator is dedicated to helping you seek treatment or refer a patient.

Solid Tumor Coordinator

 

Learn more