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.

Keywords: epidemiology; local recurrences; metastasis; myxofibrosarcoma; prognostic factors.

Figure 1

Kaplan–Meier curve showing the survival of MFS patients diagnosed in 2002–2010 in comparison to patients that were diagnosed in 2011–2019.

Figure 2

Overall survival of MFS patients with recurrence/metastasis as calculated from date of first diagnosis. (a) Kaplan–Meier curves showing the difference in survival with and without local recurrences, (b) Kaplan–Meier curves showing the difference in survival with and without regional recurrences, (c) Kaplan–Meier curves showing the difference in survival with and without distant recurrences.