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
Review
. 2022 Apr;11(8):1805-1816.
doi: 10.1002/cam4.4580. Epub 2022 Feb 17.

Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review

Elizabeth A Connolly  1   2   3 , Vivek A Bhadri  1   2   3   4 , Johnathon Wake  4 , Katrina M Ingley  5 , Jeremy Lewin  5   6   7 , Susie Bae  5   7 , Daniel D Wong  8 , Anne P Long  9 , David Pryor  10 , Stephen R Thompson  11   12 , Madeleine C Strach  1   2   3 , Peter S Grimison  1   2   3 , Annabelle Mahar  13 , Fiona Bonar  14 , Fiona Maclean  14 , Angela Hong  1   2   3
Affiliations
Review

Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review

Elizabeth A Connolly et al. Cancer Med. 2022 Apr.

Abstract

CIC-rearranged sarcoma is a recently established, ultra-rare, molecularly defined sarcoma subtype. We aimed to further characterise clinical features of CIC-rearranged sarcomas and explore clinical management including systemic treatments and outcomes.

Methods: A multi-centre retrospective cohort study of patients diagnosed between 2014-2019.

Results: Eighteen patients were identified. The median age was 27 years (range 13-56), 10 patients were male (56%), 11 patients (61%) had localised disease and 7 patients had advanced (metastatic or unresectable) disease at diagnosis. Of 11 patients with localised disease at diagnosis, median overall survival (OS) was 40.6 months and the 1-, 2- and 5-year OS estimates were 82%, 64% and 34% respectively. Nine patients (82%) underwent surgery (all had R0 resections), 8 (73%) patients received radiotherapy to the primary site (median dose 57Gy in 28 fractions), and 8 (73%) patients received chemotherapy (predominantly Ewing-based regimens). Metastases developed in 55% with a median time to recurrence of 10.5 months. In patients with advanced disease at diagnosis, median OS was 12.6 months (95% CI 5.1-20.1), 1-year OS was 57%. Median progression-free survival was 5.8 months (95% CI 4.5-7.2). Durable systemic therapy responses occurred infrequently with a median duration of systemic treatment response of 2.1 months. One durable complete response of metastatic disease to VDC/IE chemotherapy was seen. Responses to pazopanib (n = 1) and pembrolizumab (n = 1) were not seen.

Conclusion: In this series, CIC-rearranged sarcomas affected young adults and had a high incidence of presenting with, or developing, metastatic disease. The prognosis overall was poor. In advanced disease, durable systemic therapy responses were infrequent.

Keywords: CIC; DUX4; ewing-like sarcoma; rearrangement; round cell sarcoma; ultra-rare sarcoma.

PubMed Disclaimer

Conflict of interest statement

The authors declare that there is no conflict of interest regarding the publication of this article.

Figures

FIGURE 1
FIGURE 1
Kaplan–Meier curves of overall survival of CIC‐rearranged sarcoma
FIGURE 2
FIGURE 2
Kaplan–Meier curves of progression free survival of CIC‐rearranged sarcoma
See this image and copyright information in PMC

References

    1. Stacchiotti S, Frezza AM, Blay JY, et al. Ultra‐rare sarcomas: a consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities. Cancer. 2021;127:2934‐2942. - PMC - PubMed
    1. Antonescu CR, Owosho AA, Zhang L, et al. Sarcomas with CIC‐rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases. Am J Surg Pathol. 2017;41(7):941‐949. - PMC - PubMed
    1. Board WCoTe . WHO Classification of Tumours of Soft Tissue and Bone, Vol. 3. 5th ed. IARC; 2020.
    1. Kinnaman MD, Zhu C, Weiser DA, et al. Survey of paediatric oncologists and pathologists regarding their views and experiences with variant translocations in ewing and ewing‐like sarcoma: a report of the children's oncology group. Sarcoma. 2020;2020:3498549‐9. - PMC - PubMed
    1. Brady EJ, Hameed M, Tap WD, Hwang S. Imaging features and clinical course of undifferentiated round cell sarcomas with CIC‐DUX4 and BCOR‐CCNB3 translocations. Skelet Radiol. 2021;50(3):521‐529. - PMC - PubMed

Publication types