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

Introduction. A GP may only encounter one sarcoma in their professional career. Early diagnosis and treatment will improve the outcomes of this rare and malignant disease. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005. These advise and assist in the early referral of potential bone and soft tissue sarcoma. Prior to 2000 only ~10% of GP referrals were proven to be malignant. Methods. In a referral region of ∼3m, we reviewed the referral patterns of suspected sarcoma by General Practitioners since 2005 in an effort to determine whether the published NICE guidelines had influenced an improvement in the diagnosis and management of malignant disease. Results. 33.3% of all referrals made by General Practitioners using the Thames Valley Cancer Network referral proforma were malignant. Discussion. NICE sarcoma guidelines for GPs have been effective in improving the diagnosis of malignant disease with suspected sarcoma albeit as a triage for malignancy rather than specifically for sarcoma

Introduction. General Practitioners will be confronted with a sarcoma rarely in their working lives. Most will never see a bone sarcoma although most will see at least one soft tissue sarcoma. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005, containing basic guidance on the earlier diagnosis of bone and soft tissue tumours. Referral criteria include: masses greater than 5cm in diameter, masses deep to fascia, masses which are fixed or immobile, masses which are painful, those which are increasing in size and recurrence after previous excision. Methods. We examined 350 General Practitioner referrals over the past 3 years to our service and examined the correlation between the above criteria and the likelihood of malignancy. Results. 33.3% of all tumours were malignant. The likelihood of a painful tumour proving malignant was 33.1% and therefore pain was not a valid predictor of malignancy (p = 0.86). Effectively, pain did not confer a relative risk. The likelihood of a growing tumour proving malignant was 38.5% and therefore increasing size is a predictor of malignancy (p = 0.03). Increasing size conferred a relative risk of 1.42 on a growing tumour. 67% of GP's said that they felt uncertain in determining whether a lesion was deep to fascia. Discussion. Whilst the introduction of the NICE guidelines has provided a clearer indication for referral than existed previously, we did not find all the referral criteria to be of particular value. In particular, pain did not correlate strongly with the likelihood of a tumour proving malignant. Specifically, GPs need further education to improve their working knowledge of this rare disease

Background. The National Institute of Clinical Excellence (NICE) published clinical guidelines in 2006 defining urgent referral criteria for soft tissue sarcoma to help improve the diagnostic accuracy and overall outcome. Despite these guidelines inadvertent excisions of soft tissue sarcomas continue to occur with alarming frequency potentially compromising patient outcomes. Objective. We reviewed the East Midlands Sarcoma Service experience of treating inadvertent excision of STSs and highlight the patient profile, referral pattern, subsequent management and oncological outcome associated with inadvertent resection. Methods. Patients were identified from our sarcoma database and a retrospective case note review performed. Results. Over a period of 32 months, 42 patients presented to our specialist centre after unplanned excision of soft tissue sarcomas. There were 29 men and 13 women, with a mean age at presentation of 59 years (19-90). 50% of the tumours were located in lower extremity, 33% around the trunk and 17% in the upper extremity. The unplanned surgery was most commonly from general surgeons, non-specialist orthopaedic surgeons, general practitioners followed by plastic surgeons. Re-resection was undertaken in 40 (95.2%) cases to achieve clear margins. Residual tumour was present in 74% of cases. Resected specimen histology was high grade in 90% of cases. Limb salvage surgery was not possible in 4 cases. Conclusion. Unplanned excision of sarcoma by non-oncologic surgeons remains a problem. It appears that it is equally prevalent in varied surgical community and general practitioners. Patients with soft tissue masses of unknown identity should be appropriately imaged and if the diagnosis remains unclear be transferred to centres that specialize in treating sarcomas for biopsy and adequate initial resection. Implementation of NICE guidelines and local strategies could improve the expedient management of these patients

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.

Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%).

Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18).

Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.

We have reviewed the data from our regional Bone Tumour Registry on patients with osteosarcoma diagnosed between 1933 and 2004 in order to investigate the relationship between survival and changes in treatment. There were 184 patients with non-metastatic appendicular osteosarcoma diagnosed at the age of 18 or under. Survival was calculated using Kaplan-Meier curves, and multivariate analysis was performed using the Cox regression proportional hazards model.

The five-year survival improved from 21% between 1933 and 1959, to 62% between 1990 and 1999. During this time, a multi-disciplinary organisation was gradually developed to manage treatment. The most significant variable affecting outcome was the date of diagnosis, with trends in improved survival mirroring the introduction of increasingly effective chemotherapy. Our experience suggests that the guidelines of the National Institute for Clinical Excellence on the minimum throughput of centres for treatment should be enforced flexibly in those that can demonstrate that their historical and contemporary results are comparable to those published nationally and internationally.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay.

Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis.

Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.