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Objectives: The optimal frequency and modality of sarcoma surveillance imaging are uncertain, and current practices vary substantially. While efforts to develop evidence-based guidelines are ongoing, patient perspectives regarding surveillance imaging have not been reported. The primary goal of this study was to pilot the novel Sarcoma Surveillance Survey to assess patient concerns regarding sarcoma surveillance., Methods: In this single-center, cross-sectional study, patients receiving surveillance imaging after surgical sarcoma treatment were administered the 10-item Sarcoma Surveillance Survey, the validated Appraisal Scale, measuring positive and negative emotional reactions to imaging, and the Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety Short Form 8a as a measure of anxiety., Results: Patients expressed highest levels of concern about cost and radiation exposure associated with surveillance, and most (87.6%) did not express a preference for more or less frequent imaging. Younger patients and those living further away from the imaging center were more concerned about cost of surveillance. Female patients had higher levels of concern compared to males regarding radiation, IV contrast, and overall levels of concern about surveillance. Higher levels of anxiety were correlated with preference for more frequent imaging (r s  = 0.274, p = 0.027) and higher overall level of concern about surveillance (r s  = 0.259, p = 0.037). Higher negative appraisal scores were also correlated with higher overall concerns (r s  = 0.323; p = 0.012)., Conclusions: Patient perspectives should be considered when developing sarcoma surveillance strategies. Identifying patients with greater anxiety and concerns regarding imaging may create opportunities for improved surveillance practices as well as counseling and survivorship interventions., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to report., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background and Objectives: Functional outcomes are important for oncology patients undergoing lower extremity reconstruction. The objective of the current study was to describe patient reported function after surgery and identify predictors of postoperative function in musculoskeletal oncology patients undergoing lower extremity endoprosthetic reconstruction., Methods: We performed a cohort study with functional outcome data from the recently completed Prophylactic Antibiotic Regimens in Tumor Surgery (PARITY) trial. We utilized the 100-point Toronto Extremity Salvage Score (TESS), which was administered pre-operatively and at 3, 6 and 12 months post-operatively. Higher scores indicate better physical functioning, and the minimally important difference is 11 points. We calculated mean functional scores at each timepoint after surgery and developed a logistic regression model to explore predictors of failure to achieve excellent post-operative function (TESS ≥ 80) at 1 year after surgery., Results: The 555 patients included in our cohort showed important functional improvement from pre-surgery to 1 year post-surgery (mean difference 14.9 points, 95%CI 12.2 to 17.6; p < 0.001) and 64% achieved excellent post-operative function. Our adjusted regression model found that poor (TESS 0-39) pre-operative function (odds ratio [OR] 3.3, 95%CI 1.6 to 6.6); absolute risk [AR] 24%, 95%CI 8% to 41.2%), older age (OR per 10-year increase from age 12, 1.32, 95%CI 1.17, 1.49; AR 4.5%, 95%CI 2.4% to 6.6%), and patients undergoing reconstruction for soft-tissue sarcomas (OR 2.3, 95%CI 1.03 to 5.01; AR 15.3%, 95%CI 0.4% to 34.4%), were associated with higher odds of failing to achieve an excellent functional outcome at 1-year follow-up. Patients undergoing reconstruction for giant cell tumors were more likely to achieve an excellent functional outcome post-operatively (OR 0.40, 95%CI 0.17 to 0.95; AR -9.9%, 95%CI -14.4% to -0.7%)., Conclusions: The majority of patients with tumors of the lower extremity undergoing endoprosthetic reconstruction achieved excellent function at 1 year after surgery. Older age, poor pre-operative function, and endoprosthetic reconstruction for soft tissue sarcomas were associated with worse outcomes; reconstruction for giant cell tumors was associated with better post-operative function., Level of Evidence: Therapeutic Level IV.

Objectives: The COVID-19 pandemic is unprecedented as a global crisis over the last century. How do specialist surgeons make decisions about patient care in these unprecedent times?, Design: Between April and May 2020, we conducted an international qualitative study. Sarcoma surgeons from diverse global settings participated in 60 min interviews exploring surgical decision making during COVID-19. Interview data were analysed using an inductive thematic analysis approach., Setting: Participants represented public and private hospitals in 14 countries, in different phases of the first wave of the pandemic: Australia, Argentina, Canada, India, Italy, Japan, Nigeria, Singapore, Spain, South Africa, Switzerland, Turkey, UK and USA., Participants: From 22 invited sarcoma surgeons, 18 surgeons participated. Participants had an average of 19 years experience as a sarcoma surgeon., Results: 17/18 participants described a decision they had made about patient care since the start of the pandemic that was unique to them, that is, without precedence. Common to 'unique' decisions about patient care was uncertainty about what was going on and what would happen in the future ( theme 1: the context of uncertainty ), the impact of the pandemic on resources or threat of the pandemic to overwhelm resources ( theme 2: limited resources ), perceived increased risk to self ( theme 3: duty of care ) and least-worst decision making, in which none of the options were perceived as ideal and participants settled on the least-worst option at that point in time ( theme 4: least-worst decision making )., Conclusions: In the context of rapidly changing standards of justice and beneficence in patient care, traditional decision-making frameworks may no longer apply. Based on the experiences of surgeons in this study, we describe a framework of least-worst decision making. This framework gives rise to actionable strategies that can support decision making in sarcoma and other specialised fields of surgery, both during the current crisis and beyond., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To determine the proportion of patients with extremity sarcoma who would be willing to participate in a clinical trial in which they would be randomised to one of four different postoperative sarcoma surveillance regimens. Additionally, we assessed patients' perspectives on the burden of cancer care, factors that influence comfort with randomisation and the importance of cancer research., Design: Prospective, cross-sectional patient survey., Setting: Outpatient sarcoma clinics in Canada, the USA and Spain between May 2017 and April 2020. Survey data were entered into a study-specific database., Participants: Patients with extremity sarcoma who had completed definitive treatment from seven clinics across Canada, the USA and Spain., Main Outcome Measures: The proportion of patients with extremity sarcoma who would be willing to participate in a randomised controlled trial (RCT) that evaluates varying postoperative cancer surveillance regimens., Results: One hundred thirty complete surveys were obtained. Respondents reported a wide range of burdens related to clinical care and surveillance. The majority of patients (85.5%) responded that they would agree to participate in a cancer surveillance RCT if eligible. The most common reason to participate was that they wanted to help future patients. Those that would decline to participate most commonly reported that participating in research would be too much of a burden for them at a time when they are already feeling overwhelmed. However, most patients agreed that cancer research will help doctors better understand and treat cancer., Conclusions: These results demonstrate that most participants would be willing to participate in an RCT that evaluates varying postoperative cancer surveillance regimens. Participants' motivation for trial participation included altruistic reasons to help future patients and deterrents to trial participation included the overwhelming burden of a cancer diagnosis. These results will help inform the development of patient-centred RCT protocols in sarcoma surveillance research., Level of Evidence: V., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background Context: Spinal sarcomas are a rare, heterogeneous group of mesenchymal tumors. Current literature reporting demographic variables and survival information is limited to small case series, and a single registry with variable treatment modalities and time periods., Purpose: We report on population-level data regarding all spinal sarcomas diagnosed over a 23-year period in Ontario, Canada, for the purposes of calculating incidence and prevalence of these tumors. Secondarily, survival is assessed by tumor type as well as adjuvant therapies during this time period., Study Design: Retrospective Cohort Study PATIENT SAMPLE: Population-based data from the Institute for Clinical Evaluative Sciences (ICES) between 1993 and 2015., Outcome Measures: Outcome measures include incidence and prevalence of spinal osteosarcoma, Ewing's sarcoma, and chondrosarcoma of the spine, as well as 2-, 5-, 10- and 15-year survival and prevalence of adjuvant therapies., Methods: Utilizing population-based data from the Institute for Clinical Evaluative Sciences (ICES) between 1993 and 2015, ICD codes were searched and available data extracted for the purposes of reporting basic demographic information and calculation of Kaplan Meyer survival curves. Databases include the Ontario Cancer Registry, Discharge Abstract Database, Ontario Health Insurance Plan, National Ambulatory Care Reporting System, Registered Persons DataBase (death) were analyzed., Results: One hundred and seven spinal sarcomas were identified, with a mean incidence was 0.38 sarcomas per million population per year, that was stable over time. The mean prevalence was 8.1 sarcomas per million population. The most common diagnosis was Ewing's sarcoma (48 [44.9%] patients), followed by chondrosarcoma (33 [30.8%] patients), and osteosarcoma (26 [24.3%] patients). Chondrosarcoma had the highest survival rates with 77.2% and 64.2% 5- and 10-year survival rates, respectively, followed by Ewing's sarcoma with 48.1% and 44.9% 5 and 10-year survival and osteosarcoma with 36.0% and 30.9% 5- and 10-year survival., Conclusions: Spinal sarcoma is a rare disease with variable survival depending on the histologic diagnosis. This population-level study involves a heterogeneous group of patients with variable stages of disease at presentation and variable treatments. Our data fit with the published literature for survival for those treated conservatively and surgically. Our data show considerable improvement in 5- and 10-year mortality when compared with previous population level studies on earlier patient cohorts, likely reflecting improvements in systemic and surgical treatments., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Aims: To make recommendations on the indications for molecular testing regarding the diagnosis, prediction of prognosis, and treatment selection in adult patients with s oft tissue sarcomas (STS) excluding gastrointestinal stromal tumour., Materials and Methods: This guideline was developed by the Cancer Care Ontario's Program in Evidence-Based Care (PEBC) and the Sarcoma Disease Site Group (DSG). The medline, embase, and Cochrane Library databases, main guideline websites, abstracts of relevant annual meetings, and PROSPERO databases were searched (January 2005 to October 2016). Internal and external reviews were conducted, with final approval by the PEBC and the Sarcoma DSG., Results: Based on the available evidence, we made three S trong Recommendations, 14 Recommendations, 9 Qualified Statements, and seven No Recommendations. The three Strong Recommendations include: i) MDM2 amplification by fluorescence in situ hybridization (FISH) is recommended as a sensitive and specific test to differentiate patients with atypical lipomatous tumour/well-differentiated liposarcoma, or dedifferentiated liposarcoma from lipoma or other STS in the differential diagnosis; ii) SS18 (SYT) break-apart by FISH or SS18-SSX (SYT-SSX) fusion by reverse transcription-polymerase chain reaction is recommended as a sensitive and specific test to differentiate patients with synovial sarcoma from other sarcomas; iii) CTNNB1 S45F mutation by polymerase chain reaction is recommended as a prognostic factor for poor recurrence-free survival in patients with desmoid tumours., Conclusion: This guideline may serve as a framework for the thoughtful implementation of molecular studies at cancer centres and other jurisdictions. Some of the recommendations may need to be updated when new evidence appears in the future., Competing Interests: Declaration of Competing Interest BCD has received molecular test kits (free of charge) from Illumina, and MG has declared that she received more than $5,000 in 2017 to act in a consulting capacity from Wright Medical and Kuros Biosciences. The other authors have declared no conflicts of interest., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: The physical function of children with sarcoma after surgery has not been studied explicitly. This paucity of research is partly because of the lack of a sufficiently sensitive pediatric functional measure. The goal of this study was to establish and validate a standardized measure of physical function in pediatric patients with extremity tumors., Questions/purposes: (1) What is the best format and content for new upper- and lower-extremity measures of physical function in the pediatric population? (2) Do the new measures exhibit floor and/or ceiling effects, internal consistency, and test-retest reliability? (3) Are the new measures valid?, Methods: In Phase 1, interviews with 17 consecutive children and adolescents with bone tumors were conducted to modify the format and content of draft versions of the pediatric Toronto Extremity Salvage Score (pTESS). In Phase 2, the pTESS was formally translated into French. In Phase 3, 122 participants between 7 and 17.9 years old with malignant or benign-aggressive bone tumors completed the limb-specific measure on two occasions. Older adolescents also completed the adult TESS. Floor and ceiling effects, internal consistency, test-retest reliability, and validity were evaluated., Results: Feedback from interviews resulted in the removal, addition, and modification of draft items, and the pTESS-Leg and pTESS-Arm questionnaires were finalized. Both versions exhibited no floor or ceiling effects and high internal consistency (α > 0.92). The test-retest reliability was excellent for the pTESS-Leg (intraclass correlation coefficient [ICC] = 0.94; 95% CI, 0.90-0.97) and good for the pTESS-Arm (ICC = 0.86; 95% CI, 0.61-0.96). Known-group validity (ability to discriminate between groups) was demonstrated by lower mean pTESS-Leg scores for participants using gait aids or braces (mean = 68; SD = 21) than for those who did not (mean = 87; SD = 11; p < 0.001). There was no significant difference between pTESS arm scores among respondents using a brace (n = 5; mean = 73; SD = 11) and those without (n = 22; mean = 83; SD = 19; p = 0.13). To evaluate construct validity, we tested a priori hypotheses. The duration since chemotherapy correlated moderately with higher pTESS-Leg scores (r = 0.4; p < 0.001) but not with pTESS-Arm scores (r = 0.1; p = 0.80), and the duration since tumor resection correlated moderately with higher pTESS-Leg scores (r = 0.4; p < 0.001) but not pTESS-Arm scores (r = 0.2; p = 0.4). Higher VAS scores (that is, it was harder to do things) antecorrelated with both pTESS versions (pTESS-Leg: r = -0.7; p < 0.001; pTESS-Arm: r = -0.8; p < 0.001). To assess criterion validity, we compared the pTESS with the current "gold standard" (adult TESS). Among adolescents, strong correlations were observed between the TESS and pTESS-Leg (r = 0.97, p < 0.001) and pTESS-Arm (r = 0.9, p = 0.007)., Conclusions: Both pTESS versions exhibited no floor or ceiling effects and had high internal consistency. The pTESS-Leg demonstrated excellent reliability and validity, and the pTESS-Arm demonstrated good reliability and reasonable validity. The pTESS is recommended for cross-sectional evaluation of self-reported physical function in pediatric patients with bone tumors., Level of Evidence: Level II, outcome measurement development.

Background: The molecular pathogenesis of many forms of soft tissue sarcomas (STS) have been rigorously characterized in the medical literature, which may be particularly important for the diagnosis and prediction of prognosis in STS., Methods: Electronic databases (2005 to October 2016) were searched. Gastrointestinal stromal tumor and pediatric sarcomas were excluded. The eligible individual study's risk of bias and the quality of aggregate evidence were assessed. Meta-analyses were performed., Results: Of 6674 identified articles, 70 were eligible and analyzed, covering 13 types of STS. Meta-analyses showed that the test of detecting MDM2 amplification by fluorescence in situ hybridization was accurate in differentiating atypical lipomatous tumor/well-differentiated liposarcoma/dedifferentiated liposarcoma from benign tumors (N = 971; sensitivity = 95%, 95% confidence interval [CI] 89-98; specificity = 100%, CI 89-100) or from other STS (N = 347; sensitivity = 99%, CI 72-100; specificity = 90%, CI 78-95); that the test of detecting SS18-SSX fusion by reverse transcription polymerase chain reaction (PCR) was accurate in differentiating synovial sarcoma from other STS (N = 532; sensitivity = 93%, CI 85-96; specificity = 99%, CI 96-100). The presence of a CTNNB1 S45F mutation detected by PCR was a risk factor for decreased recurrence-free survival in desmoid tumors (N = 418; hazard ratio from 3.50 [CI 1.51-8.14] to 6.20 [CI 2.24-17.15])., Conclusions: Sarcomas are rare cancers whose molecular pathogenesis is becoming increasingly understood. The current evidence demonstrates that molecular analyses are useful in the diagnosis and prediction of prognosis in some STS., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Introduction: Orthopaedic oncology researchers face several obstacles in the design and execution of randomised controlled trials, including finite fiscal resources to support the rising costs of clinical research and insufficient patient volume at individual sites. As a result, high-quality research to guide clinical practice has lagged behind other surgical subspecialties. A focused approach is imperative to design a research programme that is economical, streamlined and addresses clinically relevant endpoints. The primary objective of this study will be to use a consensus-based approach to identify research priorities for international clinical trials in orthopaedic oncology., Methods and Analysis: We will conduct a 3-phase modified Delphi method consisting of 2 sequential rounds of anonymous web-based questionnaires (phases I and II), and an in-person consensus meeting (phase III). Participants will suggest research questions that they believe are of particular importance to the field (phase I), and individually rate each proposed question on 5 criteria (phase II). Research questions that meet predetermined consensus thresholds will be brought forward to the consensus meeting (phase III) for discussion by an expert panel. Following these discussions, the expert panel will be asked to assign scores for each research question, and research questions meeting predetermined criteria will be brought forward for final ranking. The expert panel will then be asked to rank the top 3 research questions, and these 3 research questions will be distributed to the initial group of participants for validation., Ethics and Dissemination: An ethics application is currently under review with the Hamilton Integrated Research Ethics Board in Hamilton, Ontario, Canada. The results of this initiative will be disseminated through peer-reviewed publications and conference presentations., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: Surgical management of metastatic bone disease (MBD) is typically reserved for lesions with the highest risk of fracture. However, the high risk of perioperative complications associated with surgery may outweigh the benefits of improved pain and/or function. The goal of this study was to (1) assess the quality of current evidence in this domain; (2) confirm that surgical management of metastases to the long bones and pelvis/acetabulum provides pain relief and improved function; and (3) assess perioperative morbidity and mortality rates., Methods: We conducted a systematic review of the literature for clinical studies that reported pain relief and function outcomes, as well as perioperative complications and mortality, in patients with MBD to the long bones and/or pelvis/acetabulum treated surgically. Multiple databases were searched up to January 2012. Pooled weighted proportions are reported., Results: Forty-five studies were included in the final analysis, with 807 patients. All included studies were level IV with 'moderate' overall quality of evidence using the Methodological Index for Non-Randomized Studies scale. Pain relief following surgical management of metastases was 93, 91, and 93 % in the humerus, femur, and pelvis/acetabulum, respectively. Maintained or improved function after surgery was seen in 94, 89, and 94 % in the humerus, femur, and pelvis/acetabulum, respectively. Perioperative complications and mortality were 17 and 4 %, respectively., Conclusions: Despite the inherent limitations of the current evidence, a benefit for the surgical management of bone metastases to the long bones and pelvis/acetabulum is evident; however, there is still substantial risk of perioperative morbidity and mortality that should be considered.

Nodular fasciitis is a benign proliferation of myofibroblasts usually arising adjacent to the fascia. In this report, we describe a rare case in which nodular fasciitis occurred in an intra-articular location in the shoulder of a 26-year-old man. The mass developed in the subscapularis recess of the shoulder and histological evaluation showed a myofibroblastic proliferation. MRI findings of intra-articular nodular fasciitis are discussed along with a review of previous reports.

Background: The goal of this study was to determine the oncologic outcomes in localized resectable soft-tissue sarcoma after pre- versus postoperative radiation., Methods: Literature searches through MEDLINE, EMBASE, CancerLit, and the Cochrane Database were performed with the following search terms: sarcoma, radiation, preoperative, and postoperative. Two reviewers independently assessed all eligible publications with the Detsky Quality Scale for Randomized Trials and the Newcastle-Ottawa Quality Assessment Scale for case-control studies. The primary outcome measure was the pooled odds ratio and 95% confidence intervals (95% CI) for the risk of local recurrence calculated through the fixed- and random-effects methods. Time-dependent survival data were calculated as an average across all studies., Results: Five eligible studies were identified including a total of 1,098 patients. The P value for heterogeneity was 0.259, and the variability (I (2)) in results across studies due to true differences in treatment effect was 25%. The risk for local recurrence was lower in the preoperative group with an odds ratio of 0.61 (95% CI 0.42-0.89) by means of the fixed-effects method, and an odds ratio of 0.67 (95% CI 0.39-1.15) by means of the random-effects method. Average survival was 76% (range 62-88%) in the preoperative group and 67% (range 41-83%) in the post-operative group., Conclusions: The delay in surgical resection necessary to complete preoperative radiation does not seem to increase the risk of lethal metastatic spread. The risk of local recurrence may be lower after preoperative radiation. These findings must be interpreted with caution because of the heterogeneity and bias in the available studies.

Background: The use of adjuvant chemotherapy to treat adults with localized resectable soft-tissue sarcoma remains controversial. The objective of this systematic review was to update the 1997 meta-analysis of randomized controlled trials (RCTs) to reassess the efficacy of doxorubicin-based chemotherapy with respect to recurrence and survival., Methods: A comprehensive literature search was performed to identify RCTs of adjuvant chemotherapy for adult patients diagnosed with localized resectable soft-tissue sarcoma. Two reviewers independently assessed eligibility and quality of the studies using a modified version of the Detsky Quality Scale. The outcome measures were local, distant, and overall recurrence and survival calculated through the fixed effect or random effect model., Results: Four new eligible trials were identified allowing for a total of 18 trials representing 1953 patients to be included in the analysis. The odds ratios (OR) for local recurrence was 0.73 (95% confidence interval [CI] 0.56-0.94; P = .02) in favor of chemotherapy. For distant and overall recurrence the OR was 0.67 (95% CI 0.56-0.82; P = .0001) in favor of chemotherapy. In terms of survival, doxorubicin alone had an OR of 0.84 (95% CI, 0.68-1.03; P = .09), which as not statistically significant. However, the OR for doxorubicin combined with ifosfamide was 0.56 (95% CI, 0.36-0.85; P = .01) in favor of chemotherapy., Conclusions: This updated meta-analysis confirms the marginal efficacy of chemotherapy in localized resectable soft-tissue sarcoma with respect to local recurrence, distant recurrence, overall recurrence, and overall survival. These benefits are further improved with the addition of ifosfamide to doxorubicin-based regimens, but must be weighed against associated toxicities., ((c) 2008 American Cancer Society)

Questions 1. In limb salvage surgery for extremity soft--tissue sarcoma (STS), what is an adequate surgical margin? 2. What is the appropriate number of samples to take from the margins of a surgical resection specimen? 3. What is the appropriate handling of surgical resection specimens? Background Surgery is the primary treatment for extremity STS. The combination of radiotherapy with surgery allows for limb salvage by using radiation to biologically "sterilize" microscopic extensions of tumour and to spare neurovascular and osseous structures. Adjuvant chemotherapy in STS--except for rhabdomyosarcoma and Ewing sarcoma--continues to be controversial. Methods The MEDLINE and EMBASE databases (1975 to June 2011) and the Cochrane Library were searched for pertinent studies. The Web sites of the main guideline organizations and the American Society of Clinical Oncology conference proceedings (2007-2010) were also searched. Results and Conclusions Thirty--three papers, including four guidelines, one protocol, and one abstract, were eligible for inclusion. The data suggest that patients with clear margins have a better prognosis, but no prospective studies have indicated how wide margins should be. In limb--salvage surgery for extremity STS, the procedure should be planned to achieve a clear margin. However, to preserve functionality, surgery may result in a very close (<1 cm) or even microscopically positive margin. In this circumstance, the use of preoperative or postoperative radiation should be considered. No studies described the optimal number of tissue sections required to assess adequacy of excision nor the appropriate handling of surgical resection specimens. The Sarcoma Disease Site Group made its recommendations based on expert opinion and consensus. [ABSTRACT FROM AUTHOR]