Aim. The aim of this study was to assess the incidence of low-grade fibromyxoid sarcoma (LGFMS), present treatment results of metastatic LGFMS, and investigate the clinical significance of the FUS gene rearrangement. Methods. This study included 14 consecutive LGFMS patients treated at the Aarhus Sarcoma Centre in 1979–2010. Fluorescent in situ hybridization (FISH) analysis for FUS break-apart was performed for all patients. Results. The incidence of LGFMS was 0.18 per million, representing 0.6% of all soft tissue sarcomas. Four patients needed multiple biopsies/resections before the correct diagnosis was made. Four patients experienced local recurrence, and three patients developed metastases. The treatment of metastatic LGFMS varied from multiagent chemotherapy to repeated, selective surgery of operable metastases. The best response to chemotherapy was short-term stabilization of disease progression, seen with Trabectedin. The prevalence of the FUS break-apart was 21.4%. We found no significant difference in clinical characteristics and outcomes in correlation with the FUS break-apart. Conclusion. LGFMS is a rare disease with multiple challenges. The FUS break-apart was not associated with local recurrence or metastases in our study. To date the only treatment resulting in disease-free periods is surgery; however further investigation into the management of metastatic LGFMS is necessary.

Background and purposePrevious studies of soft tissue sarcoma (STS) have identified a number of possible prognostic factors; however, the majority of these include highly selected populations, with unclear validation of data and insufficient statistical methods. We identified prognostic factors in a validated, population-based 30-year series of STS treated at a single institution, using an advanced statistical approach.Patients and methodsBetween 1979 and 2008, 922 adult patients from western Denmark were treated at the Aarhus Sarcoma Center for non-metastatic STS in the extremities or trunk. The endpoints were local recurrence (LR) and disease-specific mortality (DSM). Prognostic factors were analyzed using a proportional hazard model, including continuous variables as cubic splines. Directed acyclic graphs were used to depict the causal structure.ResultsThe 5-year LR was 16% and the 5-year DSM was 24%. Important prognostic factors for both LR and DSM were age, duration of symptoms, tumor size, grade, margin, and radiotherapy, while anatomical location (upper, lower extremity, trunk) was prognostic for DSM.InterpretationIn this population-based series of adult, non-metastatic STS, we included directed acyclic graphs, cubic splines, and a competing risk model in order to minimize bias, and demonstrated that these statistical methods are feasible. Using these statistical methods on a large, validated dataset, we excluded depth as a prognostic factor and established that age, duration of symptoms, size, grade, margin, and radiotherapy were important prognostic factors for both local recurrence and disease-specific mortality.

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Background:The aim of the present study was to validate the data in the Aarhus Sarcoma Registry (ASR), to determine if this registry is population-based for western Denmark, and to examine the incidence of sarcomas using validated, population-based registry data.Methods:This study was based on patients with bone and soft tissue sarcoma treated at the Sarcoma Centre of Aarhus University Hospital between January 1, 1979 and December 31, 2008. The validation process included a review of all medical files by two researchers using a standardized form. The Danish Cancer Registry was used as a reference to assess the completeness of registration of patients in the ASR. Crude and World Health Organization age-standardized incidence, as well as age-, gender-, and year-specific incidences were estimated.Results:The validation process added 385 to the 1442 patients who were registered in the ASR. Before validation, on average, 70.5% of the data for the variables was correct. Validation improved the average completeness of the registered variables from 83.7% to 99.3%. The 1827 patients in the ASR after validation include 85.3% of the patients registered in the Danish Cancer Registry. The overall World Health Organization age-standardized incidence of sarcoma in the trunk or extremities in western Denmark in the period 1979–2008 was 2.2 per 100,000, being 0.8 for bone sarcomas and 1.4 for soft tissue sarcomas.Conclusion:The validation process significantly improved the completeness of the variables and the quality of the ASR data. ASR is now a valuable population-based tool for epidemiological research and quality improvement in the treatment of sarcoma. It is our recommendation that documented validation of registries should be a prerequisite for publishing studies derived from them.

background. Comorbidity is an important prognostic factor for survival in other cancers, but the importance in soft tissue sarcoma has not yet been clarified. The aims of this study were to examine the prevalence of comorbidity in soft tissue sarcoma patients, and estimate the impact of comorbidity on overall and disease-specific mortality. Material and methods. Overall, 1210 adult patients with soft tissue sarcoma in the extremities or trunk wall were identified through the Aarhus Sarcoma Registry, a validated population-based database. Information on comorbidity was obtained through the National Patient Registry, and a Charlson's Comorbidity score was calculated for each patient. The prevalence of comorbidity was assessed overall, as well as according to age and year of diagnosis. Overall and diseasespecific mortality rates according to level of comorbidity were computed. The prognostic value of comorbidity was estimated using crude and adjusted Cox proportional hazard models. results. The overall prevalence of comorbidity was 25%. The prevalence increased with increasing age, and patients with comorbidity had a larger proportion of adverse prognostic factors when compared to patients without comorbidity. The five-year disease-specific mortality was 26% (95% CI 24-29) for patients without comorbidity, compared to 33% (95% CI 24-42), 41% (95% CI 32-50), and 44% (95% CI 33-55) for patients with mild, moderate, and severe comorbidity, respectively. After adjusting for age, sex, stage, tumor size, depth, grade, surgical margin, radiotherapy, and chemotherapy, comorbidity was independently associated with an increased overall and disease-specific mortality. conclusion. Patients with comorbidity had significantly increased overall and disease-specific mortality compared to patients without comorbidity, even when adjusting for important prognostic factors including age.