GRaCefUl
Research type
Research Study
Full title
Global collaboRAtion on CIC-DUX4, BCOR-CCN3, high-grade Undifferentiated round cell sarcoma (URCS) project
IRAS ID
335151
Contact name
Sandra Strauss
Contact email
Sponsor organisation
IRCCS Istituto Ortopedico Rizzoli
Duration of Study in the UK
1 years, 5 months, 1 days
Research summary
Undifferentiated round cell sarcomas (URCS) are an extremely rare form of cancer called Sarcoma. These compromise a heterogeneous group of bone and soft tissue tumours occurring primarily in young adults (but distributed across all ages).
URCS represent a diagnostic challenge. URCS cell histology and expression mostly resemble another sub-type of Sarcoma called ‘Ewing sarcoma’ but may also may include other sub-types of Sarcomas Definitive classification usually requires detection of entity-specific mutations.
Recently, the use of modern molecular techniques led to the description of a variety of new, rare, sarcoma subtypes, which have morphological appearance similar to Ewing sarcomas (called "Ewing-like" sarcomas) but carry gene fusions different from that of Ewing sarcoma. The identification of these gene fusions suggests that other, yet to be identified, gene fusions could be associated with this type of tumour.
This study proposes to collect data from all cases of URCSs treated within the referral centres for sarcoma in order to reclassify and describe their treatment and outcome.
This is a multicentre retrospective and prospective study that will analyse all cases with a diagnosis of URCSs from 01 January 1983 to present, and all new cases referred to each participant site globally. The study will look at both clinical, histological and molecular (laboratory) data collected. Clinical data such treatments, outcomes, and prognostic factors and correlate with molecular analysis to reclassify these subtypes.
Available archival tissue samples (FFPE Blocks) will have laboratory analysis performed as per local standards and processes for molecular re-classification. Molecular analysis will be by real-time PCR and/or FISH analysis, or other relevant analysis.
Any laboratory analysis data, such as genomic/genetic characteristics, where available will also be collected to correlate with clinical outcomes.In the UK, no laboratory analysis of samples will take place, and only historic laboratory analysis data (FISH, real-time PCR, NGS and RNA-Seq (genomic/genetic)) obtained from standard of care procedures will be collected.
REC name
London - Surrey Borders Research Ethics Committee
REC reference
26/PR/0374
Date of REC Opinion
15 May 2026
REC opinion
Further Information Favourable Opinion