Backgrounds and Objectives This investigation sought to describe the outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS). Methods This was a review of the Surveillance, Epidemiology, and End Results database from 1975 to 2016. Kaplan‐Meier methods were used to estimate disease‐specific survival (DSS), and a Cox regression model was used to identify prognostic factors. Results Of the 7502 identifiable cases, 1% (n = 74) were PLB and 99% (n = 7428) were SLMS. Survival was the same between PLB and SLMS (p = .209). On multivariable analysis for high‐grade SLMS, radiation (neoadjuvant: hazard ratio [HR], 0.56; 95% confidence interval [CI], 0.4–0.8; p = .003; adjuvant: HR, 0.75; 95% CI, 0.6–0.9; p = .008) and surgery (procedure specific) improved DSS. For PLB, wide resection/limb salvage (HR, 0.40; 95% CI, 0.3–0.5; p = .018) and amputation (HR, 0.69; 95% CI, 0.5–0.9; p < .001) were positive prognostic factors. Neither radiation nor chemotherapy were prognostic factors for survival in PLB. Conclusions For SLMS, radiation portends a survival advantage. For PLB, however, neither chemotherapy nor radiation were significant prognostic factors, which suggests the optimal treatment for PLB, similar to other primary soft tissue sarcomas originating in bone, remains an unmet medical need.

Background and Objectives: While historically aggressive, some synovial sarcomas (SS) are clinically indolent. This study sought to determine whether SS grade predicts oncologic outcomes and whether Grade 1 disease might exist.Methods: Thirty-five cases from 2010 to 2019 were retrospectively reviewed.Clinicopathological data were analyzed and Kaplan-Meier assessed survival. Results:The median patient age was 37 years (interquartile range: 28-51.5). The local control rate was 74.3%, and recurrence-free survival (RFS) was worse in positive versus negative margin resections (p = 0.023). The incidence of metastasis was 21.9% (n = 7) at a median 31 ± 31.7 months, and metastasis-free survival was 50.0% in Grade 3 SS versus 86.5% in Grade 2 (p = 0.026). Among a theoretical Grade 1 group, the overall survival (OS) and RFS profiles were improved compared to Grade 2 and 3 SS, respectively (p = 0.014 and p = 0.030). The Grade 1 group had a 15.8% (n = 3) metastatic rate and 80% 10-year survival.Conclusions: Tumor grade appears to predict outcomes in SS. A theoretical Grade 1 group showed improved OS and RFS versus Grades 2 and 3 SS, with metastatic rates and long-term survival resembling the historical literature for other low-grade soft tissue sarcomas. Our group continues to support the French Federation of Cancer Centers diagnostic strategy and NCCN treatment guidelines for SS.

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Brain metastases in sarcoma are exceedingly rare, with few published series documenting ranges from 1% to 8%. This study investigated the outcomes of sarcoma patients with brain metastases using a population-based analysis. This was a retrospective review of 5933 patients with high-grade sarcoma identified from the Surveillance, Epidemiology, and End Results database between 2010 and 2015. Of the eligible 5933 patients, 0.7% ( n = 44) had brain metastasis. Kaplan–Meier was used to estimate survival and follow-up (reverse Kaplan–Meier), and a multivariable Cox proportional hazards model analyzed prognostic factors of disease-free survival (DFS). Median (IQR) follow-up of all eligible patients was 28 months (12; 47). Patients who developed brain metastasis had a higher proportion of N1 stage disease ( p < 0.001), as well as synchronous metastasis to bones, liver, and lungs compared to those without brain metastasis (all p < 0.001). The median (IQR) DFS with brain metastasis was 6 months (2; 12), and survival with brain metastasis was significantly worse than DFS in patients without brain metastasis ( p < 0.001). Among those with brain metastasis only, there was no difference in DFS with respect to sex, race, primary tumor origin, T stage or N stage disease, synchronous metastasis to bone, liver or lung, nor with respect to chemotherapy or radiation for treatment of the primary tumor (all p > 0.05). For sarcoma patients with brain metastasis, the outcomes are poor and do not appear to differ by clinicopathologic factors. However, patients with certain histologies and synchronous metastases may warrant more frequent surveillance as there was an association of brain metastasis with these factors.

Background and ObjectivesMyxofibrosarcoma (MFS) is an aggressive soft tissue tumor with an unpredictable recurrence pattern. We sought to (a) determine whether margin status in MFS is correlated to rates of local recurrence (LR) and (b) identify demographic and treatment variables associated with disease‐related outcomes in this population.MethodsThis retrospective study identified 42 surgically treated patients with MFS over 10 years at a single institution. Patient demographics, tumor characteristics, intraoperative variables, and disease‐related outcomes were recorded.ResultsThirty‐three (83%) patients had negative surgical margins and seven (18%) had positive margins. Four of 32 patients (13%) with negative margins developed subsequent LR compared to six of seven (86%) patients with positive margins (p < .001). Three patients (75%) with metastatic disease were deceased at the end of the study, while five (15%) without metastasis were deceased (p = .024).ConclusionsPositive margin procedures for MFS were associated with LR. However, negative surgical margins demonstrated a relatively high rate of LR compared to other soft tissue sarcoma subtypes. Furthermore, though MFS tends to locally recur and have a propensity for distant metastasis, patients are observed to have a higher probability of death from other causes.