Regional variation in the survival of patients with a soft-tissue sarcoma of the extremity and trunk wall under a centralized care system : what has been the impact of national policies in the UK?

AIMS: While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. METHODS: The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management. RESULTS: There had been a significant difference in survival between patients referred from the North East, North West, East Midlands, West Midlands, South West, and Wales in the pre-NICE era (five-year disease-specific survival (DSS); South West, 74% vs North East, 47% (p = 0.045) or West Midlands, 54% (p = 0.049)), which was most evident for patients with a high-grade STS. However, this variation disappeared in the post-NICE era, in which the overall DSS for high-grade STS improved from 47% to 68% at five years (p < 0.001). Variation in the size of the tumour closely correlated with the variation in DSS, and the overall size of the tumour and incidence of metastasis at the time of diagnosis also decreased after the national policies were issued. CONCLUSION: The survival of patients with a STS improved and regional variation corrected after the introduction of national policies, as a result of a decreasing size of tumour and incidence of metastasis at the time of diagnosis, particularly in patients with a high-grade STS. This highlights the positive impact of national guidelines on regional variation in the presentation, management, and outcome in patients with a STS. Cite this article: Bone Joint J 2021;103-B(9):1541-1549.

Impact of NICE guidelines on the survival of patients with soft-tissue sarcomas.

AIMS: Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. METHODS: A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated. RESULTS: The mean size of the tumour was significantly smaller at the time of diagnosis (10.3 cm (SD 6.5) vs 9.1 cm (SD 6.2); p < 0.001) and the number of patients who had undergone an inadvertent excision significantly decreased (28% (n = 389) vs 20% (n = 204); p < 0.001) following the introduction of the NICE guidelines. The five-year DSS was 63% in the pre-NICE and 71% in post-NICE groups (p < 0.001). The improved survival was more significant for those with a high-grade tumour (pre-NICE, 48%; post-NICE, 68%; p < 0.001). In those with a high-grade tumour, the mean size of the tumour (11.6 cm (SD 6.2) vs 9.6 cm (SD 5.8); p < 0.001) and the number of patients with metastasis at the time of diagnosis (15% (n = 124 vs 10% (n = 80); p = 0.007) significantly decreased in the post-NICE group. CONCLUSION: An improvement in survival was seen after the introduction of the NICE guidelines, especially in patients with a high-grade STS. More patients were referred at an earlier stage, indicating a clearer pathway after the issue of national policy for the management of STSs in the UK. Cite this article: Bone Joint J 2021;103-B(3):569-577.

Outcome of Ewing's sarcoma of the scapula-a long-term follow-up study.

OBJECTIVE: The aim of this study was to review our experience of managing Ewing's sarcoma of scapula and to identify features predictive of a good outcome. HYPOTHESIS: Surgery and neo-adjuvant chemotherapy would have a better survival than surgery alone. PATIENTS AND METHODS: Between 1988 and 2018, 29patients, (14females and 15males) were treated at a single hospital: The median age at diagnosis was 17.6years (range: 2 to 51years). Of the 29cases, 3 were primary soft tissue Ewing's invading bone and remainder were bony sarcoma. Twenty three patients underwent surgery and 6 patients were managed non-operatively. All surgical resection specimens were classified according to Musculoskeletal Tumour Society based on surgical margins assessed by specialist musculoskeletal pathologists. The function was assessed using the Musculoskeletal Tumour Society Score. RESULTS: The median follow-up for all patients was 10 years (range: 2 months to 30 years) and for survivors, median follow-up was 13.7 years (range: 18months to 30 years). The estimated disease-specific survival for all patients was 71.4% (95% CI 84.6-50.7%) at five years and 63% (95% CI 78.3-41.9%) at ten years. The estimated disease-specific survival for patients that underwent surgery was 86.5% (95% CI 95.5-63.7%) at five years and 81% (95% CI 92.5-56.9%) at ten years. The median MSTS score was 67.4%. CONCLUSION: In this, the largest single series of patients with an Ewing's sarcoma of the scapula treated in a single hospital, those treated with surgery and neo-adjuvant chemotherapy had the best chance for survival compared to chemotherapy and/or radiotherapy alone. Local recurrence did not have an affect on survival. LEVEL OF EVIDENCE: IV, retrospective study.

Secondary chondrosarcoma arising from osteochondroma: outcomes and prognostic factors.

AIMS: The aim of this study was to report the outcomes of patients who underwent definitive surgery for secondary chondrosarcomas arising from osteochondromas. PATIENTS AND METHODS: A total of 51 patients with secondary chondrosarcomas occurring from osteochondromas were reviewed. Median age was 36 years (interquartile range (IQR) 15 to 82). Median follow-up was 6.9 years (IQR 2.8 to 10.6). The pelvis was the most commonly affected site (59%). Histological grades were grade I in 35 (69%), grade II in 13 (25%), and grade III in three patients (6%). RESULTS: Preoperative biopsy histology correctly predicted the final histological grade in 27% of patients. The ten-year disease-specific survival (DSS) for all patients was 89.4%. Local recurrence occurred in 15 patients (29%), more commonly in pelvic tumours (37%) compared with limb tumours (19%). Four patients with pelvic tumours died from progression of local recurrence. No patient with limb tumours died of disease. Wide/radical margin was associated with improved local recurrence-free survival (p = 0.032) and local recurrence was associated with worse DSS (p = 0.005). CONCLUSION: We recommend that a secondary chondrosarcoma arising from osteochondroma of the pelvis is resected with wide/radical resection margins. The balance between the morbidity of surgery and risk of local recurrence needs to be considered in patients with limb secondary chondrosarcomas. Cite this article: Bone Joint J 2019;101-B:1313-1320.

Synovial sarcoma: Do children do better?

OBJECTIVES: Synovial sarcoma, a distinct subtype of soft tissue sarcomas (STS), is typically found in young patients. Long history of symptoms and heterogeneous clinical presentation sometimes delays diagnosis. Children have been reported to have a better prognosis than adults in some series. The main emphasis of this study was to determine differences between children and adults and to investigate prognostic factors regarding cancer specific survival (CSS). METHODS: 248 patients treated between 1982 and 2014 at one department were included. Mean age was 37.0 years, including 43 patients <16 years. Demographic, pathology- and treatment-related information was ascertained. Median follow-up was 5.2 years. RESULTS: Median duration of symptoms was 11.5 months in children and 12 months in adults (p = 0.238). Patients with a prior unplanned excision had a significantly longer duration of symptoms (p = 0.001). No difference was present between children and adults regarding tumour size, site, grade and superficial/deep location. Treatment was with surgical excision and (usually) adjuvant radiotherapy but five patients received preoperative radiotherapy and 43 patients chemotherapy. In patients treated with curative intent, five-year CSS rates were 75.5% for adults and 89.0% for children, with 10-year CSS rates of 56.1% and 82.2% (p = 0.026). In multivariate analysis, large tumour size (p < 0.005) and patient age (p = 0.024) were associated with worse CSS, irrespective of tumour location and site. CONCLUSION: Clinical presentation of synovial sarcoma is similar in children and adults, with no significant difference in tumour size, site, grade or location. Small tumour size and young patient age are independent positive prognostic factors influencing CSS.