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Overall Survival of Patients with Myxofibrosarcomas: An Epidemiological Study

Authors: Chiel A. J. van der Horst; Sabien L. M. Bongers; Yvonne M. H. Versleijen-Jonkers; Vincent K. Y. Ho; Pètra M. Braam; Uta E. Flucke; Johannes H. W. de Wilt; +1 Authors

Overall Survival of Patients with Myxofibrosarcomas: An Epidemiological Study

Abstract

Myxofibrosarcoma (MFS) is a rare mesenchymal soft tissue sarcoma type, with a high local recurrence (LR) rate. Robust epidemiological data on MFS are lacking. We, therefore, aimed to identify prognostic factors and describe real-life outcomes of a large cohort of 908 MFS patients obtained from the nationwide database of the Netherlands Cancer Registry and diagnosed between 2002 and 2019. Median Overall survival (OS) was 155 (range 0.1–215) months, with a five-year OS of 67.7%. No improvement of OS was found over time. Multivariable Cox regression survival analysis demonstrated known prognostic factors for OS, such as older age, tumour size, and histological grade with the addition of sex. Surgery at sarcoma expertise centres, instead of general hospitals, was associated with better OS outcomes. In a subcohort of 177 patients, 39% developed LR with a median time to recurrence of 20 months. From LR on, the median OS was 64.0 months (CI 95% 38.5–89.5). In 28%, distant metastases were diagnosed with a median OS of 34.3 months (CI 95% 28.8–39.8) after diagnosis of the primary tumour. In this largest nationwide cohort so far, survival outcomes and recurrence rates for MFS patients did not improve over time, emphasizing the need to improve treatment strategies and suggesting a role for sarcoma expertise centres.

Country
Netherlands
Keywords

myxofibrosarcoma; local recurrences; metastasis; prognostic factors; epidemiology, Radiation Oncology - Radboud University Medical Center, Pathology - Radboud University Medical Center, Radboud University Medical Center, Radboudumc 9: Rare cancers RIHS: Radboud Institute for Health Sciences, Radboudumc 14: Tumours of the digestive tract RIHS: Radboud Institute for Health Sciences, Radboudumc 9: Rare cancers RIMLS: Radboud Institute for Molecular Life Sciences, Medical Oncology - Radboud University Medical Center, Article, Surgery - Radboud University Medical Center

  • BIP!
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    citations
    This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
    23
    popularity
    This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
    Top 10%
    influence
    This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
    Top 10%
    impulse
    This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
    Top 10%
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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
23
Top 10%
Top 10%
Top 10%
Green
gold