Metastatic Ewing Sarcoma Treatment Study
The purpose of this study is to compare the eventfree survival (EFS) in patients with newly diagnosed metastatic Ewing sarcoma treated with multiagent chemotherapy with and without the addition of ganitumab (AMG 479).
Also, the study will describe the toxicity of the addition of ganitumab to multimodality therapy for patients with newly diagnosed metastatic Ewing sarcoma.
In order to participate you must meet the following criteria:
Must be ≤ 50 years of age at enrollment of this study.
Note: Infants and small children are eligible for this study, however, the treating physicians and family must be prepared to deliver adequate local control as required in this study.
Have a histologic diagnosis (by institutional pathologist) of newly diagnosed Ewing sarcoma or peripheral primitive neuroectodermal tumor (PNET) arising from bone or soft tissue and with metastatic disease involving lung, bone, bone marrow, or other metastatic site.
For the purpose of this study metastatic disease is defined as:
Lesions which are discontinuous from the primary tumor, are not regional lymph nodes, and do not share a bone or body cavity with the primary tumor.
Skip lesions in the same bone as the primary tumor do not constitute metastatic disease. Skip lesions in an adjacent bone are considered bone metastases. If there is any doubt whether lesions are metastatic, a biopsy of those lesions should be performed.
- Contralateral pleural effusion and/or contralateral pleural nodules.
- Distant lymph node involvement.
Patients with pulmonary nodules are considered to have metastatic disease if the patient has:
- Solitary nodule ≥ 0.5 cm or multiple nodules of ≥ 0.3 cm unless lesion is biopsied and negative for tumor
- Patients with solitary nodule < 0.5 cm or multiple nodules < 0.3 cm are not considered to have lung metastasis unless biopsy documents tumor.
- Bone marrow metastatic disease is based on morphologic evidence of Ewing sarcoma based on H&E stains. In the absence of morphologic evidence of marrow involvement on H&E, patients with bone marrow involvement detected ONLY by flow cytometry, RT-PCR, FISH, or immunohistochemistry will NOT be considered to have clinical bone marrow involvement for the purposes of this study.
- Lesions which are discontinuous from the primary tumor, are not regional lymph nodes, and do not share a bone or body cavity with the primary tumor.
Allow required bilateral bone marrow biopsies at study entry.
- The suggested approach for patients with large pelvic tumors in which a posterior iliac crest bone marrow biopsy would track through the tumor is to instead under go 2 marrow biopsies on the contralateral side (either 2 posterior biopsies or one posterior and one anterior biopsy).
Allow whole body FDG-PET scans to screen patients for bone metastases.
- Areas suspicious for bone metastasis based on FDG-PET scans require confirmatory anatomic imaging with either MRI or CT (whole body FDG-PET/CT or FDG-PET/MR scan acceptable). Whole body technetium bone scans may be performed at the discretion of the investigator and are not required. For patients without other sites of metastatic disease whose sole metastatic site to qualify for study entry is a single area suspicious for bone metastasis identified by FDG-PET, confirmatory biopsy or anatomic imaging evidence of an associated soft tissue mass at that site is required for study entry.
You will be excluded from the study if any of the following criteria apply to you:
- Have regional node involvement as your only site of disease beyond the primary tumor.
- Have primary tumors arise in the intradural soft tissue (eg. brain and spinal cord).
- Have received prior chemotherapy or radiation therapy.
This is a partial list of inclusion and exclusion criteria.