Soft Tissue Sarcomas in Adolescents and Young Adults.

Soft tissue sarcomas (STS) represent a heterogeneous group of extraskeletal mesenchymal tumors that affect individuals throughout the entire age continuum. Despite this pervasive influence, key differences exist in the presentation of these sarcomas across varying age groups that have prevented a more uniform approach to management. Notably, rhabdomyosarcoma (RMS) is more common in children, while most nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) subtypes are more prevalent in adults. Older patients with NRSTS appear to have more molecularly complex biology and often present with more advanced disease compared with children. Poorer outcome disparities are observed in older patients with RMS despite receiving similar treatment as younger patients. In this review, we highlight differences in epidemiology, biology, and management paradigms for pediatric and adult patients with STS and explore opportunities for a unified approach to enhance the care and outcomes within the AYA population.

Treatments and prognostic factors for bone and soft tissue sarcoma in non-urban areas in Japan.

BACKGROUND: Although bone and soft tissue sarcoma is recognized as a rare cancer that originates throughout the body, few comprehensive reports regarding it have been published in Japan. PATIENTS AND METHODS: Bone and soft tissue sarcomas were tabulated from the Cancer Registries at eight university hospitals in the Chugoku-Shikoku region. Prognostic factors in cases were extracted in a single facility and have been analyzed. RESULTS: From 2016 to 2019, 3.4 patients with bone and soft tissue sarcomas per a general population of 100,000 were treated at eight university hospitals. The number of patients who underwent multidisciplinary treatment involving collaboration among multiple clinical departments has been increasing recently. In the analysis carried out at a single institute (Ehime University Hospital), a total of 127 patients (male/female: 54/73) with an average age of 67.0 y (median 69.5) were treated for four years, with a 5-year survival rate of 55.0%. In the analysis of prognostic factors by multivariate, disease stage and its relative treatment, renal function (creatinine), and a patient's ability of self-judgment, and a patient's mobility and physical capability were associated with patient prognosis regarding bone and soft tissue sarcomas. Interestingly, age did not affect the patient's prognosis (> 70 vs ≦ 70). CONCLUSIONS: Physical and social factors may affect the prognosis of patients with bone and soft tissue sarcomas, especially those living in non-urban areas.

Sex Differences in Soft Tissue Sarcoma: Incidence, Clinicopathological Profile, Survival, and Costs.

Background: There are evident sex differences in the incidence of and mortality rates for several tumors. Soft tissue sarcomas (STSs) account for no more than 1% of all malignancies in adults. This study aimed to provide a comprehensive overview of the sex differences in the epidemiology of STSs and the related costs. Methods: This retrospective population-based study draws on epidemiological data regarding cases of STS collected by the cancer registry of the Italian Veneto region for the years 1990-2018. A joinpoint regression analysis was performed to identify significant changes in the trends of the standardized incidence rates in males and females. Bivariate and survival analyses were conducted to assess differences in clinicopathological characteristics and short-term mortality by sex. Direct health care costs incurred over 2 years after a diagnosis of STS were calculated, stratified by sex. Results: The incidence rates of STS at any age were higher for males; only among males the incidence rates showed a tendency to slightly increase. No significant sex differences came to light in short-term mortality or clinicopathological profile, except for the cancer site. Health care costs in the 2 years after a diagnosis of STS were not sex related. Conclusion: The STS incidence was found to be higher for males and showed a rising trend over the last three decades only for males. These findings could result from the occupational exposure to environmental mutagens mainly involving men. Sex did not affect the survival or the clinicopathological STS profile.

Clinical features and outcomes of infantile soft-tissue sarcoma: A multicenter retrospective study in Beijing.

Background: Soft-tissue sarcomas during infancy are rare and understudied. With no data on this specific condition, we performed a retrospective study of infant-onset sarcomas based on a multi-institutional cohort in Beijing, China, collected over the past decade. We reviewed infantile soft-tissue sarcomas' clinical characteristics, treatments, and outcomes. Materials and Methods: The patients with soft-tissue sarcoma diagnosed from 0 to 12 months in four primary children's hospitals in Beijing from January 2010 to December 2019 were evaluated. Results: Fifty-one patients were enrolled, including 31 males and 20 females. The median age at the diagnosis was five months (range, 0-12), and seven (13.7%) patients were diagnosed in the first month of their life. Histologically, twenty-five patients were diagnosed with rhabdomyosarcoma (RMS), six were diagnosed with extraosseous Ewing sarcoma (EES), and twenty were diagnosed with nonrhabdomyosarcoma soft-tissue sarcoma (NRSTS). The treatment principles and details of RMS focused on reference to the Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols. For EES and NRSTS, chemotherapy was prescribed according to children's oncology group protocols. The five-year EFS/OS rates of RMS were 26.4% ± 19.5%/56.2 ± 17.8%, the five-year EFS/OS rate of EES was 50% ± 20.4%, and the five-year EFS/OS of NRSTS was 85.2% ± 9.8%/100%. Conclusions: Infant-onset soft-tissue sarcoma is heterogeneous. The primary location of the abdominal or pelvic cavity of RMS and EWS was at a later stage and had a poorer prognosis. Multimodal therapy resulted in successful disease control for the majority of patients. Standardization of treatment protocols will facilitate care for such challenging conditions.

Health related Quality of Life over time in German sarcoma patients. An analysis of associated factors - results of the PROSa study.

Introduction: Sarcomas are rare cancers and very heterogeneous in their location, histological subtype, and treatment. Health-Related Quality of Life (HRQoL) of sarcoma patients has rarely been investigated in longitudinal studies. Methods: Here, we assessed adult sarcoma patients and survivors between September 2017 and February 2020, and followed-up for one year in 39 study centers in Germany. Follow-up time points were 6 (t1) and 12 months (t2) after inclusion. We used a standardized, validated questionnaire (the European Organisation for Research and Treatment of Cancer Quality of Life Core Instrument (EORTC QLQ-C30) and explored predictors of HRQoL in two populations (all patients (Analysis 1), patients in ongoing complete remission (Analysis 2)) using generalized linear mixed models. Results: In total we included up to 1111 patients at baseline (915 at t1, and 847 at t2), thereof 387 participants were in complete remission at baseline (334 at t1, and 200 at t2). When analyzing all patients, HRQoL differed with regard to tumor locations: patients with sarcoma in lower extremities reported lower HRQoL values than patients with sarcomas in the upper extremities. Treatment which included radiotherapy and/or systemic therapy was associated with lower HRQoL. For patients in complete remission, smoking was associated with worse HRQoL-outcomes. In both analyses, bone sarcomas were associated with the worst HRQoL values. Being female, in the age group 55-<65 years, having lower socioeconomic status, and comorbidities were all associated with a lower HRQoL, in both analyses. Discussion: HRQoL increased partially over time since treatment and with sporting activities. HRQoL improved with time since treatment, although not in all domains, and was associated with lifestyle and socioeconomic factors. Bone sarcomas were the most affected subgroup. Methods to preserve and improve HRQoL should be developed for sarcoma patients.

Investigation of the Incidence and Geographic Distribution of Bone and Soft Tissue Sarcomas in Canada: A National Population-Based Study.

Sarcomas are a heterogeneous group of mesenchymal malignancies with various genetic and environmental risk factors. This study analyzed the epidemiology of sarcomas to gain insight into the incidence and mortality rates of these cancers in Canada, as well as to elucidate their potential environmental risk factors. Data for this study were obtained from le Registre Québécois du Cancer (LRQC) and from the Canadian Cancer Registry (CCR) for the period from 1992 to 2010. Mortality data were obtained from the Canadian Vital Statistics (CVS) database for the period from 1992 to 2010 using the International Classification of Diseases for Oncology, ICD-O-3, ICD-9, or ICD-10 codes, for all subtypes of sarcomas. We found that the overall sarcoma incidence in Canada decreased during the study period. However, there were select subtypes with increasing incidence. Peripherally located sarcomas were found to have lower mortality rates compared to axially located sarcomas, as expected. Clustering of Kaposi sarcoma cases in self-identified LGBTQ+ communities and in postal codes with a higher proportion of African-Canadian and Hispanic populations was observed. Forward Sortation Area (FSA) postal codes with a lower socioeconomic status also had higher Kaposi sarcoma incidence rates.

Is the Number of National Database Research Studies in Musculoskeletal Sarcoma Increasing, and Are These Studies Reliable?

BACKGROUND: Large national databases have become a common source of information on patterns of cancer care in the United States, particularly for low-incidence diseases such as sarcoma. Although aggregating information from many hospitals can achieve statistical power, this may come at a cost when complex variables must be abstracted from the medical record. There is a current lack of understanding of the frequency of use of the Surveillance, Epidemiology, and End Results (SEER) database and the National Cancer Database (NCDB) over the last two decades in musculoskeletal sarcoma research and whether their use tends to produce papers with conflicting findings. QUESTIONS/PURPOSES: (1) Is the number of published studies using the SEER and NCDB databases in musculoskeletal sarcoma research increasing over time? (2) What are the author, journal, and content characteristics of these studies? (3) Do studies using the SEER and the NCDB databases for similar diagnoses and study questions report concordant or discordant key findings? (4) Are the administrative data reported by our institution to the SEER and the NCDB databases concordant with the data in our longitudinally maintained, physician-run orthopaedic oncology dataset? METHODS: To answer our first three questions, PubMed was searched from 2001 through 2020 for all studies using the SEER or the NCDB databases to evaluate sarcoma. Studies were excluded from the review if they did not use these databases or studied anatomic locations other than the extremities, nonretroperitoneal pelvis, trunk, chest wall, or spine. To answer our first question, the number of SEER and NCDB studies were counted by year. The publication rate over the 20-year span was assessed with simple linear regression modeling. The difference in the mean number of studies between 5-year intervals (2001-2005, 2006-2010, 2011-2015, 2016-2020) was also assessed with Student t-tests. To answer our second question, we recorded and summarized descriptive data regarding author, journal, and content for these studies. To answer our third question, we grouped all studies by diagnosis, and then identified studies that shared the same diagnosis and a similar major study question with at least one other study. We then categorized study questions (and their associated studies) as having concordant findings, discordant findings, or mixed findings. Proportions of studies with concordant, discordant, or mixed findings were compared. To answer our fourth question, a coding audit was performed assessing the concordance of nationally reported administrative data from our institution with data from our longitudinally maintained, physician-run orthopaedic oncology dataset in a series of patients during the past 3 years. Our orthopaedic oncology dataset is maintained on a weekly basis by the senior author who manually records data directly from the medical record and sarcoma tumor board consensus notes; this dataset served as the gold standard for data comparison. We compared date of birth, surgery date, margin status, tumor size, clinical stage, and adjuvant treatment. RESULTS: The number of musculoskeletal sarcoma studies using the SEER and the NCDB databases has steadily increased over time in a linear regression model (ß = 2.51; p < 0.001). The mean number of studies per year more than tripled during 2016-2020 compared with 2011-2015 (39 versus 13 studies; mean difference 26 ± 11; p = 0.03). Of the 299 studies in total, 56% (168 of 299) have been published since 2018. Nineteen institutions published more than five studies, and the most studies from one institution was 13. Orthopaedic surgeons authored 35% (104 of 299) of studies, and medical oncology journals published 44% (130 of 299). Of the 94 studies (31% of total [94 of 299]) that shared a major study question with at least one other study, 35% (33 of 94) reported discordant key findings, 29% (27 of 94) reported mixed key findings, and 44% (41 of 94) reported concordant key findings. Both concordant and discordant groups included papers on prognostic factors, demographic factors, and treatment strategies. When we compared nationally reported administrative data from our institution with our orthopaedic oncology dataset, we found clinically important discrepancies in adjuvant treatment (19% [15 of 77]), tumor size (21% [16 of 77]), surgery date (23% [18 of 77]), surgical margins (38% [29 of 77]), and clinical stage (77% [59 of 77]). CONCLUSION: Appropriate use of databases in musculoskeletal cancer research is essential to promote clear interpretation of findings, as almost two-thirds of studies we evaluated that asked similar study questions produced discordant or mixed key findings. Readers should be mindful of the differences in what each database seeks to convey because asking the same questions of different databases may result in different answers depending on what information each database captures. Likewise, differences in how studies determine which patients to include or exclude, how they handle missing data, and what they choose to emphasize may result in different messages getting drawn from large-database studies. Still, given the rarity and heterogeneity of sarcomas, these databases remain particularly useful in musculoskeletal cancer research for nationwide incidence estimations, risk factor/prognostic factor assessment, patient demographic and hospital-level variable assessment, patterns of care over time, and hypothesis generation for future prospective studies. LEVEL OF EVIDENCE: Level III, therapeutic study.

Incidence and survival of soft tissue sarcoma in England between 2013 and 2017, an analysis from the National Cancer Registration and Analysis Service.

There is a paucity of population-based data detailing the incidence and survival of patients with soft tissue sarcoma (STS), in part due to the heterogeneity of disease and changes to classification. Here, the incidence and survival of all STS subtypes registered in England between 2013 and 2017 were analysed using cancer registry data held by the National Cancer Registration and Analysis Service. Age-standardised incidence rates were calculated per 1 000 000 using the 2013 European Standard Population. Net survival was computed using Brenner's alternative method, with the Ederer II estimator. Age-specific overall survival was assessed using Kaplan-Meier. The influence of age, sex, socioeconomic deprivation and diagnostic routes on survival was assessed using Cox proportional hazards modelling. In total, 19 717 patients were diagnosed with STS, an average of 3943 patients per year and representing approximately 0.8% of malignancies. The most common histological diagnoses were Gastrointestinal Stromal Tumours (GIST), leiomyosarcoma and undifferentiated sarcoma, accounting for 20.2%, 13.3% and 12.7% of all sarcomas, respectively. Five-year net survival for all malignant STS was 65.0%; and was lowest for patients with vascular tumours at 39%. Patients from most deprived cohorts had 23% greater chance of dying within 5 years than patients in least deprived areas. This population-based study has allowed us for the first time to define the incidence and survival rates of prevalent STS subtypes in England such as GIST, liposarcoma and leiomyosarcoma, as well as rare entities and groups with inferior outcome. This data is invaluable for service provision, benchmarking and addressing inequality.

Epidemiologic Survey of Myxofibrosarcoma Using Data from the Bone and Soft Tissue Tumor Registry in Japan.

BACKGROUND: Myxofibrosarcoma is a common sarcoma among older patients, with locally infiltrative behavior and a predilection for local postoperative recurrence. Some studies have reported the factors affecting prognosis, although only a few have mentioned the previous staging classification systems. This study investigated the clinical overview and prognosis of myxofibrosarcoma to determine the optimal treatment. METHODS: This retrospective study analyzed the records of 349 patients with myxofibrosarcoma in the nationwide Bone and Soft Tissue Tumor Registry in Japan from 2006 to 2015. Clinical features, treatment options, and patient outcomes were investigated. RESULTS: Ultimately, 349 patients were identified. The overall survival rates were 93.1% at 2 years and 84.3% at 5 years. A multivariate analysis was performed using the Cox proportional hazards model. The study identified four significant prognostic factors for survival: tumor size, depth, compartment status, and location. The prognostic score was calculated by summing the scores of all the factors. The overall survival rate was 69.3% at 5 years for the patients with prognostic scores of 6 or higher. Conversely, the patients with prognostic scores of 2 or lower had a survival rate of 95.6% at 5 years. CONCLUSIONS: Among myxofibrosarcomas, those larger than 5 cm, deep-seated, invaded into the external compartment, or in axial body parts were associated with a significantly worse prognosis. Adjuvant radiotherapy and chemotherapy did not contribute significantly to a better prognosis. Previous staging classification systems are impractical for prognosis prediction. Therefore, new classifications are needed. Further research on new treatment methods for patients with a poor prognosis will be crucial in the future.

Clinicopathological profile of myxoid soft tissue tumors- A retrospective study in a tertiary care hospital in South India.

Background: Myxoid soft tissue tumors are rare and diagnostically challenging group of tumors with varied biological behavior ranging from benign, locally aggressive to distantly metastasizing malignant tumors. Aims: The objectives of the study are to identify the relative frequency and distribution of myxoid soft tissue tumors among patients in a tertiary care hospital and to study the clinicopathological features of these tumors. This was a retrospective cross-sectional study conducted in the department of pathology of a tertiary care hospital from January 2008 to December 2013. Materials and Methods: Clinical and pathological details of all the 80 myxoid soft tissue tumors reported during the study period were retrieved from the records of department of pathology. Corresponding Hematoxylin & Eosin (H & E) slides were reviewed, and Immunohistochemistry (IHC) was carried out for confirmation. The relationship among various prognostic variables was analyzed in case of myxoid sarcomas. Results: Myxoid soft tissue tumors accounted for 3.7% among the soft tissue tumors with a predominance of malignant myxoid sarcomas (71.25%) in contrast to the overall picture of sarcomas. Myxoid neurofibroma (34.78%) was the most common benign tumor, while myxofibrosarcoma (33.33%) was the frequent myxoid sarcoma. A statistically significant correlation was seen between tumor size and depth (P-value: 0.038) and also between presence of vascular invasion and histological grade (P-value: 0.012) of sarcomas. Conclusion: Light microscopic morphology, supplemented by ancillary techniques like IHC, remains the cornerstone for diagnosis of myxoid soft tissue tumors.

Soft-tissue Sarcoma Survival in the US Military Health System: Comparison With the SEER Program.

INTRODUCTION: The US Military Health System (MHS) provides universal health care to beneficiaries. Few studies have evaluated the potential influence of access to universal care on survival outcomes for sarcoma. This study compared the survival of adult patients with soft-tissue sarcoma in the MHS with the US general population. METHODS: MHS data were obtained from the Department of Defense Automated Central Tumor Registry (ACTUR). US population data were obtained from the National Cancer Institute's Surveillance, Epidemiology, and End Results registry. Patients who were 25 years or older with a histologically confirmed musculoskeletal soft-tissue sarcoma were matched based on age, sex, and race. Kaplan-Meier survival curves and Cox proportional hazards models were used to compare 5-year survival in the two groups. RESULTS: Adult patients in ACTUR had markedly lower 5-year mortality for soft-tissue sarcomas (hazard ratio=0.82; 95% confidence interval, 0.73 to 0.92) after adjustment for potential confounders. Lower 5-year mortality was found in most demographic subgroups for ACTUR patients compared with Surveillance, Epidemiology, and End Results patients. CONCLUSION: Five-year survival in the MHS compared with the US general population may suggest an important role of universal health care in improving the survival of patients with soft-tissue sarcoma.

Factors Affecting Delay in Initial Treatment of Patients with Soft Tissue Sarcomas.

Background: Soft tissue sarcomas (STS) are rare, and little is known about the factors that affect the delays in the initial treatment. The aim of this study is to quantify the period between onset of symptoms and start of treatment of STS and determine the factors affecting delays in initial treatment. Methods: This is a retrospective study of all STS treated in our institution between October 2009 and March 2019. We analysed patient record to determine the period from onset of symptoms to start of initial treatment. We also collected data with regard to patient characteristics and features of the tumour. Tumours were classified into upper extremity, lower extremity, trunk and others based on location of the tumour. Statistical tests were done to identify factors that affected delay in initial treatment. Results: The study included 134 patients (76 male and 58 female) with STS with an average age of 56.6 years. The tumours involved the upper extremity in 20 patients, lower extremity and trunk in 50 patients each and other areas in 14 patients. The most frequent histological subtypes were liposarcomas (n = 31, 23.5%) and undifferentiated pleomorphic sarcomas (n = 24, 18.2%). Initial treatment was delayed by an average of 9.9 months for all groups. The period of treatment delay for tumours involving the upper extremity was shorter (7.9 months) and these tumours were smaller at initial presentation (57.6 mm) compared to tumours in other locations (p < 0.05). Other factors that were positively associated with treatment delays were a history of diabetes mellitus (p = 0.037) and smoking (p = 0.026). Conclusion: Patients with upper-extremity STS may have the benefit of a relatively better prognosis as they present earlier and with a smaller tumour. In addition, factors, such as diabetes and smoking, which indicate a low interest in health also influenced the delay in the initial treatment. Level of Evidence: Level III (Therapeutic).

Soft Tissue Sarcomas: A 16-Year Experience of a Tertiary Referral Hospital in North Jordan.

Background and Objectives: Sarcomas are rare malignant tumors of mesenchymal origin. Their low prevalence and histological heterogeneity make their diagnosis a challenging task. To the best of our knowledge, the epidemiology of soft tissue sarcomas (STSs) was not well studied in Jordan. This study thus aimed to determine STS epidemiologic trends at King Abdullah University Hospital (KAUH); a tertiary hospital that provides cancer healthcare for 70% of the population in Irbid Governorate, North Jordan. The findings of this study will provide a good reference point of the burden of STSs in Jordan and the Middle East region. Materials and Methods: All cases with confirmed STS diagnoses who attended KAUH from January 2003 until December 2018 were included in the initial analysis. Bone sarcomas, gastrointestinal stromal tumors and uterine sarcomas were not included in the study. Information collected from the pathology reports and electronic medical records was used to determine STS prevalence, incidence rate, age and gender distributions, histological types and anatomic location. Cases were reviewed by three pathologists with interest in soft tissue tumors. The findings were compared with literature. Results: In total, 157 STS cases were reported (1.9% of cancers diagnosed at KAUH during the 16-year study period). Crude annual incidence rate (IR) per 100,000 person-years ranged from 0.48 in 2015 to 1.83 in 2011 (average = 1.04). Age-standardized IR (ASR)(World WHO 2000-2025) was 1.37. Male:female ratio was 1.3:1. Median age was 39 years. Age ranged from <1 year to 90 years. Overall STS rates increased with age. The most common histological types were liposarcoma (19%), rhabdomyosarcoma (17%) and leiomyosarcoma (10%). The most common anatomic location was the extremity (40.1%), followed by the trunk (14.7%), then head and neck (10.8%). Conclusion: STSs are rare in North Jordan. A slight increase in their incidence was identified during the study period similar to global trends. The collection of relevant data on established risk factors along with a broader scale evaluation of the epidemiology of STS in the Middle East region is recommended to better evaluate disease burden and trends.

Prevalence of elastofibroma dorsi found incidentally upon chest computed tomography scan: A tertiary care center experience.

OBJECTIVES: To determine the prevalence and demographic data regarding incidentally encountered elastofibroma dorsi (EFD) in the Saudi population-based on chest CT (computed tomography) scans. METHODS: This retrospective study was carried out on 4,435 chest CT examinations that were collected between January 2014 and December 2016 in Riyadh, Saudi Arabia. One musculoskeletal radiologist who was unaware of the patients' clinical presentations or reasons for CT study reviewed the 4,435 chest CTs prospectively, searching for soft tissue tumors in the subscapular region. RESULTS: Elastofibroma dorsi was incidentally confirmed via CT scan in 36 of the 4,435 patients, including 8 males and 28 females, with a mean age of 76.6 years, yielding a prevalence of 0.8%. Elastofibroma dorsi was more common in females, with a 3.3-fold higher prevalence (77.8%) as compared to males (22.2%); however, this difference was not statistically significant (p>0.05). Twenty-five (69.44%) patients had unilateral EFD and 11 (30.56%) had bilateral EFD. Right-sided EFD was encountered in 60% of females and 50% of males. Furthermore, there was no statistically significant correlation between the size of EFD and the patient's gender or age (p>0.05). CONCLUSION: Elastofibroma dorsi is a rare but uncommon incidental finding on routine chest CT examination encountered in 0.8% of elderly Saudi patients.

Score for the Risk and Overall Survival of Lung Metastasis in Patients First Diagnosed With Soft Tissue Sarcoma: A Novel Nomogram-Based Risk Assessment System.

Background: Metastatic soft tissue sarcoma (STS) patients have a poor prognosis with a 3-year survival rate of 25%. About 30% of them present lung metastases (LM). This study aimed to construct 2 nomograms to predict the risk of LM and overall survival of STS patients with LM. Materials and Methods: The data of patients were derived from the Surveillance, Epidemiology, and End Results database during the period of 2010 to 2015. Logistic and Cox analysis was performed to determine the independent risk factors and prognostic factors of STS patients with LM, respectively. Afterward, 2 nomograms were, respectively, established based on these factors. The performance of the developed nomogram was evaluated with receiver operating characteristic curves, area under the curve (AUC) calibration curves, and decision curve analysis (DCA). Results: A total of 7643 patients with STS were included in this study. The independent predictors of LM in first-diagnosed STS patients were N stage, grade, histologic type, and tumor size. The independent prognostic factors for STS patients with LM were age, N stage, surgery, and chemotherapy. The AUCs of the diagnostic nomogram were 0.806 in the training set and 0.799 in the testing set. For the prognostic nomogram, the time-dependent AUC values of the training and testing set suggested a favorable performance and discrimination of the nomogram. The 1-, 2-, and 3-year AUC values were 0.698, 0.718, and 0.715 in the training set, and 0.669, 0.612, and 0717 in the testing set, respectively. Furthermore, for the 2 nomograms, calibration curves indicated satisfactory agreement between prediction and actual survival, and DCA indicated its clinical usefulness. Conclusion: In this study, grade, histology, N stage, and tumor size were identified as independent risk factors of LM in STS patients, age, chemotherapy surgery, and N stage were identified as independent prognostic factors of STS patients with LM, these developed nomograms may be an effective tool for accurately predicting the risk and prognosis of newly diagnosed patients with LM.

Impact of the national sarcoma guidelines on the prevalence and outcome of inadvertent excisions of soft tissue sarcomas: An observational study from a UK tertiary referral centre.

OBJECTIVES: This study aims to investigate the impact of the national guideline on the prevalence and outcome in patients with soft-tissue sarcoma (STS) who had undergone inadvertent excisions. METHODS: A total of 2336 patients were referred to a tertiary sarcoma centre from six regions (North East, North West, East Midlands, West Midlands, Wales, and South West) in the United Kingdom with a diagnosis of STS between 1996 and 2016, of whom 561 patients (24.0%) had undergone inadvertent excisions. Patients were categorised into two groups of 10-year periods pre and post the National Institute for Health and Clinical Excellence (NICE) guideline implementation in 2006. RESULTS: The proportion of inadvertent excisions decreased after the NICE guideline implementation: 27.2% (pre-NICE) versus 19.8% (post-NICE) (p = 0.001). A substantial regional variation (17.4%-34.5%) in the proportion of inadvertent excisions in the pre-NICE era was reduced in the post-NICE era (14.3%-22.4%). The 5-year disease-specific survival was 77.7% (pre-NICE) versus 75.6% (post-NICE) (p = 0.961) and there was a trend toward lower incidence of local recurrence in the post-NICE era; 13.5% (pre-NICE) versus 10.5% (post-NICE) (p = 0.522). Multivariate analyses revealed that residual tumours in re-resection specimens were independently associated with an increased risk of disease-specific mortality (HR, 3.35; p < 0.001) and local recurrence (HR, 1.99; p = 0.017), which was significantly reduced after the NICE guideline implementation (53.2% versus 42.0%; p = 0.022). CONCLUSIONS: The NICE guideline implementation reduced the proportion of patients with STS who had undergone inadvertent excisions and residual tumour in re-resection specimens, indicating an improved pre-referral management of STSs.

Benign Hand Tumors (Part II): Soft Tissue Tumors.

Background: Benign soft-tissue tumors of the hand are more common than both their benign bone and malignant soft-tissue counterparts. This study evaluates the characteristics and treatment of benign soft tissue tumors in light of 1 institution's experience. Methods: Histologically confirmed benign soft-tissue tumors of the hand were retrospectively identified using International Classification of Disease codes from 1992 to 2015. A medical chart review was conducted to collect patient demographics, tumor epidemiology, and treatment. Results: A total of 199 soft-tissue tumors were identified. The median patient age at time of treatment was 47.4 ± 14.7 years in age. The majority of tumors were located in the digits (n = 168, 84%) and treated by excision (n = 191, 96%). Localized type tenosynovial giant cell tumors (n = 71, 36%) were the most common and had the highest rates of recurrence (8.5%) in this series. Other frequent histologies included hemangioma, schwannoma, and glomus tumors. Conclusion: Awareness and understanding of tumor characteristics may help physicians with diagnosis and treatment. There is an extensive variety of tumors, but the principles of clinical and imaging diagnosis are common to all of them. Marginal excision for the treatment pain, improvement of function, and cosmetic correction applies to all these tumors independent of the histology.

The incidence of unplanned excision in patients with soft tissue sarcoma: Reports from the Bone and Soft Tissue Tumor registry in Japan.

BACKGROUND: Due to their rarity, soft tissue sarcomas (STS)s are often treated surgically as unplanned excisions (UE)s before referral to specialists. The Bone and Soft Tissue Tumor (BSTT) registry is a nationwide, organ-specific cancer registry for bone and soft-tissue tumors in Japan. The aim of this study was to elucidate the incidence and risk factors for UEs using data from the BSTT registry. METHODS: Our study comprised 8761 patients with STS including 4826 males and 3935 females, with a mean age of 59 years (range, 0-105 years). Patients were classified into primary STS and UE groups. RESULTS: Superficial and deep tumors were found in 2533 and 6120 patients, respectively. The mean STS size was 8.6 cm (range, 0.2-49 cm). We identified 6610 patients with high-grade, and 1758 patients with low-grade STS. Of the total 8761 patients, 7770 patients had primary STSs, whereas 991 patients were referred after undergoing UE. The UE mean incidence rate over 11 years was 11.3%. The UE incidence rates for leiomyosarcoma (LMS) (odds ratio [OR] = 1.75, p < 0.0001), fibrosarcoma (FS) (OR = 2.28, p < 0.0001), and epithelioid sarcoma (OR = 1.82, p = 0.01) were higher than the mean UE incidence rate (11.3%). Of 757 patients who underwent UE, 354 (47%) patients had superficial (≤5 cm) STSs, whereas 156 (20.6%) of patients had deep STSs (>5 cm) (p < 0.0001). Logistic regression analysis revealed that UE was frequently associated with males and small, superficial, and trunk STSs. CONCLUSIONS: In the present study, the mean UE incidence rate (11.3%) was significantly different from the individual UE incidence rates of histological diagnoses suggesting that surgeons should consider STS, even with small tumors. Continuous education during training for medical students and surgeons is essential to ensure awareness and correct diagnostic procedures for STS.