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

Summary

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.

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

Interventions

RADIATION

radiation

If the participant meets the criteria, participants will receive radiation therapy. Chemotherapy will continue during radiation.

DRUG

vincristine

Dosage and route of administration: Infants \< 12 months of age: 0.05 mg/kg IV day 1; participants ≥ 12 months of age: 1.5 mg/m\^2 IV day 1 (max. dose 2 mg).

DRUG

doxorubicin

Dosage and route of administration: Infants \< 1 year 2.5 mg/kg continuous infusion (CI) over 48 hours, days 1-2; participants \> 1 year of age 75 mg/m\^2 CI over 48 hours, days 1-2.

DRUG

cyclophosphamide

Dosage and route of administration: The dose and route are different in neo-adjuvant/adjuvant chemotherapy and maintenance therapy. Please see the Detailed Description for further information.

DRUG

ifosfamide

Dosage and route of administration: Infants \< 1 year of age 60 mg/kg/day IV over 60 minutes days 1-5; participants \> 12 months of age 1800 mg/m\^2 IV over 60 minutes x 5 days, days 1-5.

DRUG

etoposide

Dosage and route of administration: Infants \< 1 year of age 3.3 mg/kg/day IV over 60 minutes days 1-5; children \> 1 year 100 mg/m\^2 daily IV over 60 minutes days 1-5.

DRUG

temozolomide

Dosage and route of administration: Temozolomide 100 mg/m\^2 PO once daily, days 1-5.

DRUG

temsirolimus

Dosage and route of administration: Temsirolimus 35 mg/m\^2 IV once day 1 and day 8.

DRUG

bevacizumab

Dosage and route of administration: Bevacizumab 15 mg/kg IV on day 1 every 3 weeks.

DRUG

sorafenib

Dosage and route of administration: 90 mg/m\^2/dose PO BID

PROCEDURE

surgery

If participant meets the criteria, they will have surgical resection of their tumor.

Sponsors & Collaborators

• University of Tennessee

  collaborator OTHER

• University of Florida

  collaborator OTHER

• Nemours Children's Clinic

  collaborator OTHER

• St. Jude Children's Research Hospital

  lead OTHER

Principal Investigators

• Sara M. Federico, MD · St. Jude Children's Research Hospital

Study Design

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Model

PARALLEL

Eligibility

Max Age

25 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2013-12-27

Primary Completion

2015-08-31

Completion

2026-07-31

FDA Drug

Yes

Countries

• United States

Study Locations