Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

This protocol will study treatment for Ewing sarcoma family of tumors (ESFT) and desmoplastic small round cell tumor (DSRCT). Participants with ESFT will be divided into two treatment groups, A or B, based on tumor characteristics. Group A (standard risk) participants have tumor that is not in the pelvis, has not spread to other parts of the body, and are less than 14 years of age. Because previous clinical trials have shown that standard treatment is very effective for children whose tumors have these characteristics, these participants will receive standard treatment. Group B (high risk) participants are 14 years of age or older or have tumor in the pelvis, or the tumor has spread to other parts of the body. Participants with DSRCT in the abdomen and/or pelvis or with tumor that cannot be removed by surgery alone or has spread to other parts of the body will be included in Group B. Participants in this group are considered high risk because there is a greater chance of tumor recurring following standard treatments currently in use. All participants will be followed and evaluated for 10 years following completion of therapy.

NCT Number	NCT01946529
Lead Sponsor	St. Jude Children's Research Hospital Collaborators: University of Tennessee, University of Florida, Nemours Children's Clinic
Conditions	Desmoplastic Small Round Cell Tumor, Ewing Sarcoma of Bone or Soft Tissue, Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Enrollment	24 participants
Start Date	2013-12-27
Primary Completion	2015-08 (estimated)
Study Completion	2026-07 (estimated)
Updated on ClinicalTrials.gov	2026-02-23