The Effectiveness of “Two-Week” Referrals for Suspected Bone and Soft Tissue Sarcoma

The two-week “wait” target introduced in 2000 requires that patients with suspected cancer referred by general practitioners should be seen within two weeks. We reviewed patients who had been referred under this standard to the North of England Bone and Soft Tissue Tumour Service, to determine if the referral guidelines had been followed, and what proportion of patients referred under the guideline had malignant tumours. 40 patients were referred under the guideline between January 2004 and December 2005. Ten of these patients (2548%) had malignant tumours, compared with 243 of 507 (48%) of those referred from other sources. In 9 of the 40 cases, the patient did not meet the criteria for urgent referral. Although this target has focussed attention on shortening the time to diagnosis and treatment, prioritising patients referred from general practitioners has the potential to disadvantage those with malignant tumours referred from other sources

UK guidelines for the management of soft tissue sarcomas

Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further data and knowledge. The development of appropriate guidance, by an experienced panel referring to the evidence available, is therefore a useful foundation on which to build progress in the field. These guidelines are an update of the previous version published in 2010 (Grimer et al. in Sarcoma 2010:506182, 2010). The original guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group (BSG) and were intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. This current version has been updated and amended with reference to other European and US guidance. There are specific recommendations for the management of selected subtypes of disease including retroperitoneal and uterine sarcomas, as well as aggressive fibromatosis (desmoid tumours) and other borderline tumours commonly managed by sarcoma services. An important aim in sarcoma management is early diagnosis and prompt referral. In the UK, any patient with a suspected soft tissue sarcoma should be referred to one of the specialist regional soft tissues sarcoma services, to be managed by a specialist sarcoma multidisciplinary team. Once the diagnosis has been confirmed using appropriate imaging, plus a biopsy, the main modality of management is usually surgical excision performed by a specialist surgeon. In tumours at higher risk of recurrence or metastasis pre- or post-operative radiotherapy should be considered. Systemic anti-cancer therapy (SACT) may be utilized in some cases where the histological subtype is considered more sensitive to systemic treatment. Regular follow-up is recommended to assess local control, development of metastatic disease, and any late-effects of treatment. For local recurrence, and more rarely in selected cases of metastatic disease, surgical resection would be considered. Treatment for metastases may include radiotherapy, or systemic therapy guided by the sarcoma subtype. In some cases, symptom control and palliative care support alone will be appropriate

Feasibility of using low-cost markerless motion capture for assessing functional outcomes after lower extremity musculoskeletal cancer surgery

\ua9 2024 Furtado et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Background Physical limitations are frequent and debilitating after sarcoma treatment. Markerless motion capture (MMC) could measure these limitations. Historically expensive cumbersome systems have posed barriers to clinical translation. Research question Can inexpensive MMC [using Microsoft KinectTM] assess functional outcomes after sarcoma surgery, discriminate between tumour sub-groups and agree with existing assessments? Methods Walking, unilateral stance and kneeling were measured in a cross-sectional study of patients with lower extremity sarcomas using MMC and standard video. Summary measures of temporal, balance, gait and movement velocity were derived. Feasibility and early indicators of validity of MMC were explored by comparing MMC measures i) between tumour sub-groups; ii) against video and iii) with established sarcoma tools [Toronto Extremity Salvage Score (TESS)), Musculoskeletal Tumour Rating System (MSTS), Quality of lifecancer survivors (QoL-CS)]. Statistical analysis was conducted using SPSS v19. Tumour sub-groups were compared using Mann-Whitney U tests, MMC was compared to existing sarcoma measures using correlations and with video using Intraclass correlation coefficient agreement. Results Thirty-four adults of mean age 43 (minimum value—maximum value 19–89) years with musculoskeletal tumours in the femur (19), pelvis/hip (3), tibia (9), or ankle/foot (3) participated; 27 had limb sparing surgery and 7 amputation. MMC was well-tolerated and feasible to deliver. MMC discriminated between surgery groups for balance (p<0.05*), agreed with video for kneeling times [ICC = 0.742; p = 0.001*] and showed moderate relationships between MSTS and gait (p = 0.022*, r = -0.416); TESS and temporal outcomes (p = 0.016* and r = -0.0557*), movement velocity (p = 0.021*, r = -0.541); QoL-CS and balance (p = 0.027*, r = 0.441) [* = statistical significance]. As MMC uncovered important relationships between outcomes, it gave an insight into how functional impairments, balance, gait, disabilities and quality of life (QoL) are associated with each other. This gives an insight into mechanisms of poor outcomes, producing clinically useful data i.e. data which can inform clinical practice and guide the delivery of targeted rehabilitation. For example, patients presenting with poor balance in various activities can be prescribed with balance rehabilitation and those with difficulty in movements or activity transitions can be managed with exercises and training to improve the quality and efficiency of the movement. Significance In this first study world-wide, investigating the use of MMC after sarcoma surgery, MMC was found to be acceptable and feasible to assess functional outcomes in this cancer population. MMC demonstrated early indicators of validity and also provided new knowledge that functional impairments are related to balance during unilateral stance and kneeling, gait and movement velocity during kneeling and these outcomes in turn are related to disabilities and QoL. This highlighted important relationships between different functional outcomes and QoL, providing valuable information for delivering personalised rehabilitation. After completing future validation work in a larger study, this approach can offer promise in clinical settings. Low-cost MMC shows promise in assessing patient’s impairments in the hospitals or their homes and guiding clinical management and targeted rehabilitation based on novel MMC outcomes affected, therefore providing an opportunity for delivering personalised exercise programmes and physiotherapy care delivery for this rare cancer

Follow up after Primary Treatment of Soft Tissue Sarcoma: A Survey of Current Practice in the United Kingdom

Despite the clinical and financial implications, there is little evidence about how patients who have been treated for soft tissue sarcoma should be followed up. The purpose of this study was to determine current practice in the United Kingdom. 192 clinicians treating patients with soft tissue sarcoma were surveyed with a postal questionnaire enquiring about frequency and method of follow up and how patients would be followed up in each of 3 clinical scenarios: a patient with a trunk or extremity tumour at low risk of relapse; a patient with a trunk or extremity tumour at high risk of relapse; and a patient with a retroperitoneal or abdominal tumour. 155 (81%) clinicians responded. Clinic visits and X-rays were the most frequently used methods of follow up. Chest CT scans, local site imaging, and blood tests were used infrequently. The intensity and methods of follow up varied with each of the clinical scenarios. There was a seven-to-twenty fold variation in cost between the least and the most expensive regimes. Respondents were generally supportive of the development of the clinical trial in this area

Psychological adaptation and recovery in youth with sarcoma: a qualitative study with practical implications for clinical care and research

Objectives: This study explored factors that play a role in psychological adaptation and recovery of young people with sarcoma. / Design: Qualitative study. / Setting: National Health Service hospitals in the UK. / Methods: Using purposive sampling, participants were recruited for semistructured interviews over the telephone or face to face in order to answer questions about how cancer impacted various domains of their life. Data were analysed using a framework approach. / Results: Thirty participants, aged 15–39 years with primary sarcoma diagnosis provided in-depth accounts of their experience. Emerging themes from the interviews were grouped into two overarching themes that relate to one’s adaptation to illness: individual level and environmental level. The qualitative nature of our study sheds light on meaningful connections between various factors and their role in one’s psychological adaptation to sarcoma. We devised a visual matrix to illustrate how risk and protective factors in adaptation vary between and within individuals. / Conclusions: This study demonstrates that young people with sarcoma report an array of both positive and negative factors related to their illness experience. The route to recovery is a multifactorial process and a one-size-fits-all approach to psychosocial care proves inadequate. We propose that moving beyond the latent constructs of resilience and psychopathology towards a dynamic model of psychological adaptation and recovery in this population can result in optimisation of care. We offer some recommendations for professionals working with young people with sarcoma in clinic and research

Current approaches to management of bone sarcoma in adolescent and young adult patients

Bone tumors are a group of histologically diverse diseases that occur across all ages. Two of the commonest, osteosarcoma (OS) and Ewing sarcoma (ES), are regarded as characteristic adolescent and young adult (AYA) cancers with an incidence peak in AYAs. They are curable for some but associated with unacceptably high rates of treatment failure and morbidity. The introduction of effective new therapeutics for bone sarcomas is slow, and to date, complex biology has been insufficiently characterized to allow more rapid therapeutic exploitation. This review focuses on current standards of care, recent advances that have or may soon change that standard of care and challenges to the expert clinical research community that we suggest must be met

FOS Expression in Osteoid Osteoma and Osteoblastoma: A Valuable Ancillary Diagnostic Tool

Osteoblastoma and osteoid osteoma together are the most frequent benign bone-forming tumor, arbitrarily separated by size. In some instances, it can be difficult to differentiate osteoblastoma from osteosarcoma. Following our recent description of FOS gene rearrangement in these tumors, the aim of this study is to evaluate the value of immunohistochemistry in osteoid osteoma, osteoblastoma, and osteosarcoma for diagnostic purposes. A total of 337 cases were tested with antibodies against c-FOS: 84 osteoblastomas, 33 osteoid osteomas, 215 osteosarcomas, and 5 samples of reactive new bone formation. In all, 83% of osteoblastomas and 73% of osteoid osteoma showed significant expression of c-FOS in the osteoblastic tumor cell component. Of the osteosarcomas, 14% showed c-FOS expression, usually focal, and in areas with severe morphologic atypia which were unequivocally malignant: 4% showed more conspicuous expression, but these were negative for FOS gene rearrangement. We conclude that c-FOS immunoreactivity is present in the vast majority of osteoblastoma/osteoid osteoma, whereas its expression is usually focal or patchy, in no more than 14% of osteosarcoma biopsies. Therefore, any bone-forming tumor cases with worrying histologic features would benefit from fluorescence in situ hybridization analysis for FOS gene rearrangement. Our findings highlight the importance of undertaking a thorough assessment of expression patterns of antibodies in the light of morphologic, clinical, and radiologic features

Socio-economic patterning in early mortality of patients aged 0-49 years diagnosed with primary bone cancer in Great Britain, 1985-2008

Background: Studies have shown marked improvements in survival between 1981 and 2000 for Ewing sarcoma patients but not for osteosarcoma. This study aimed to explore socio-economic patterning in early mortality rates for both tumours. Procedure: The study analysed all 2432 osteosarcoma and 1619 Ewing sarcoma cases, aged 0-49 years, diagnosed in Great Britain 1985-2008 and followed to 31/12/2009. Logistic regression models were used to calculate risk of dying within three months, six months, one year, three years and five years after diagnosis. Associations with Townsend deprivation score and its components were examined at small-area level. Urban/rural status was studied at larger regional level. Results: For osteosarcoma, after age adjustment, mortality at three months, six months and one year was associated with higher area unemployment, OR = 1·05 (95% CI 1·00, 1·10), OR = 1·04 (95% CI 1·01, 1·08) and OR = 1·04 (95% CI 1·02, 1·06) respectively per 1% increase in unemployment. Mortality at six months was associated with greater household non-car ownership, OR = 1·02 (95% CI 1·00, 1·03). For Ewing sarcoma, there were no significant associations between mortality and overall Townsend score, nor its components for any time period. For both tumours increasing mortality was associated with less urban and more remote rural areas. Conclusions: This study found that for osteosarcoma, early mortality was associated with residence at diagnosis in areas of higher unemployment, suggesting risk of early death may be socio-economically determined. For both tumours, distance from urban centres may lead to greater risk of early death

Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study

BACKGROUND: Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. PATIENTS AND METHODS: Specialist centres collaborated to report prognostic factors and outcome for 113 patients. RESULTS: Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25). CONCLUSIONS: Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease