A decade in banking Ewing sarcoma: a report from the Children’s Oncology Group

Abstract

Outcomes for patients with metastatic and recurrent Ewing sarcoma remain poor and a better understanding of the biology of this malignancy is critical to the development of prognostic biomarkers and novel therapies. Therefore, the Children’s Oncology Group (COG) has created tissue banking protocols designed to collect high quality, clinically annotated, tumor specimens that can be distributed to researchers to perform basic science and correlative investigation. Data from the COG Ewing sarcoma tissue banking protocols AEWS02B1 and its successor study AEWS07B1 were reviewed in this study. Six-hundred and thirty five patients were enrolled on AEWS02B1 and 396 patients have had tissue submitted to AEWS07B1. The average age of participation was 13.2 years. About 86% were less than 19 years old and only 6% were greater than 21 years of age at diagnosis. When compared to SEER data, approximately 18% of all cases and only 8% of all patients >20 years old diagnosed with Ewing sarcoma annually in the United States have had tumor banked. The majority of participants submitted formalin fixed, paraffin embedded, primary tumor and blood samples. In total, fresh frozen tissue was submitted for only 29% of cases. Only seven metastatic tumor samples have been collected. Although the COG has been successful in collecting tumor samples from patients newly diagnosed with Ewing sarcoma, fresh frozen tumor specimens from primary and metastatic disease are critically needed, especially from young adult patients, in order to conduct high quality basic science and translational research investigation with a goal of developing better treatments.

Retracted: Let-7a Functions as a Tumor Suppressor in Ewing's Sarcoma Cell Lines Partly by Targeting Cyclin-Dependent Kinase 6

“…Although some advances have been developed in the treatment strategies for ES, very little improvement was achieved in the 5-year survival rates. The disease-free survival of patients with over metastases is < 30%, and this rate has not experienced any improvements over the last 30 years (Borinstein et al, 2013). Thus, a better understanding of the pathological mechanisms about this malignancy is critical to the development of prognostic biomarkers and novel therapies.…”

Clinical and Biochemical Function of Polymorphic NR0B1 GGAA-Microsatellites in Ewing Sarcoma: A Report from the Children's Oncology Group

“…All tumor samples were from patients with a pathologically confirmed diagnosis of primary Ewing sarcoma who were enrolled in a large multicenter COG protocol, AEWS0031 [21],[23]. AEWS0031 was opened for enrollment on May 2001 and closed in August 2005.…”

Plasma DNA-Based Molecular Diagnosis, Prognostication, and Monitoring of Patients With EWSR1 Fusion-Positive Sarcomas

“…This is a particularly attractive aspect of this methodology as reliable acquisition of baseline tumor tissue has proven difficult in North American cooperative group ES banking studies. 34 In this study, we demonstrated the ability to identify fusion breakpoint sequences in the majority of plasma cfDNA samples, along with potentially prognostic alterations in TP53 and STAG2. Although NGS was successful at identifying fusions in the majority of baseline cfDNA samples, there were several examples in which ddPCR was able to identify a very low level of fusion positivity that was not identified by NGS.…”