Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Apr;7(4):1384-1393.
doi: 10.1002/cam4.1415. Epub 2018 Mar 13.

Genomic complexity in pediatric synovial sarcomas (Synobio study): the European pediatric soft tissue sarcoma group (EpSSG) experience

Daniel Orbach  1 , Véronique Mosseri  2 , Daniel Pissaloux  3 , Gaelle Pierron  4 , Bernadette Brennan  5 , Andrea Ferrari  6 , Frederic Chibon  7 , Gianni Bisogno  8 , Gian Luca De Salvo  9 , Camille Chakiba  7 , Nadège Corradini  10 , Véronique Minard-Colin  11 , Anna Kelsey  12 , Dominique Ranchère-Vince  3
Affiliations

Genomic complexity in pediatric synovial sarcomas (Synobio study): the European pediatric soft tissue sarcoma group (EpSSG) experience

Daniel Orbach et al. Cancer Med. 2018 Apr.

Abstract

A genomic index (GI) tool using array comparative genomic hybridization (aCGH) on tumor cells has emerged as independent prognostic factor associated with the risk of metastatic relapse in synovial sarcoma (SS). The aim was to assess GI in pediatric patients with SS, to determine its value as a prognostic factor. All pediatric/adolescent/young adults' (<25 years) with localized SS prospectively included in the European EpSSG-NRSTS05 protocol with a contributive aCGH were selected. Definition of GI was A2 /C, where A is the total number of alterations (segmental gains and losses) and C is the number of involved chromosomes on aCGH results. GI1 group corresponds to cases with no copy number alterations (flat profile, GI = 0) and GI2 group cases with at least one or more copy number alterations (rearranged profile; GI ≥ 1). Samples were available from 61 patients. The median age of the cohort was 13 years (range: 4-24). Overall, 55.7% were GI1 group, and 44.3% GI2 . After a median follow-up of 62 months (range: 0.1-112), 10 tumor events occurred and five patients died. Respectively, for GI1 versus GI2 groups, five-year event-free survival (EFS) was 93.8 ± 4.2% versus 64.9 ± 10.1% (P < 0.006) and five-year Metastatic-Free Survival (MFS) 93.8 ± 4.2% versus 72.9 ± 9.5% (P < 0.04). In multivariate analysis, GI status as adjusted for IRS group, patient age, site, and tumor size remain independent prognostic for EFS with a relative risk (RR) of 6.4 [1.3-31.9] (P < 0.01) and RR for MFS is 4.8 [0.9-25.7] (P < 0.05). Genomic complexity evaluated through GI may explain the metastatic behavior of pediatric SS.

Keywords: Adolescent; EpSSG; comparative genomic hybridization; genomic index; synovial sarcoma.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Examples of different synovial sarcoma aCGH profiles: without any alteration (A), slightly rearranged (B), and highly rearranged (C).
Figure 2
Figure 2
Flowchart of the study. SS, synovial sarcoma; pt: patient; NOS, nonother specification; MPNST, malignant peripheral nerve sheath tumor.
Figure 3
Figure 3
Penetrance plot of GI2 synovial sarcomas analyzed with aCGH (27 cases).
Figure 4
Figure 4
Overall outcome of all patients with synovial sarcoma. OS, overall survivals; EFS, event‐free survival; MFS, metastatic‐free survival.
Figure 5
Figure 5
Event‐free survival of patients with localized synovial sarcoma according to the genomic index value. EFS, event‐free survival; GI, Genomic index.
Figure 6
Figure 6
Metastatic‐free survival of patients with localized synovial sarcoma according to the genomic index value. MFS, metastatic‐free survival; GI, Genomic index.
See this image and copyright information in PMC

References

    1. Sultan, I. , Rodriguez‐Galindo C., Saab R., Yasir S., Casanova M., and Ferrari A.. 2009. Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: an analysis of 1268 patients. Cancer 115:3537–3547. - PubMed
    1. Ferrari, A. , De Salvo G. L., Brennan B., van Noesel M. M., De Paoli A., Casanova M., et al. 2014. Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann. Oncol. 26:567–572. - PubMed
    1. Orbach, D. , Mc Dowell H., Rey A., Bouvet N., Kelsey A., and Stevens M. C.. 2011. Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP‐MMT) Working Group. Pediatr. Blood Cancer 57:1130–1136. - PubMed
    1. Ferrari, A. , Bisogno G., Alaggio R., Cecchetto G., Collini P., Rosolen A., et al. 2008. Synovial sarcoma of children and adolescents: the prognostic role of axial sites. Eur. J. Cancer 44:1202–1209. - PubMed
    1. Ferrari, A. , Gronchi A., Casanova M., Meazza C., Gandola L., Collini P., et al. 2004. Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101:627–634. - PubMed

Publication types