Materiały źródłowe

• #1 The Epidemiology of Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3564705/

  Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend 0.001). A recent meta-analysis reported that children with a history of hernias have a greater risk of developing Ewing’s sarcoma (adjusted OR 3.2, 95% CI 1.9, 5.7). Bone development during pubertal growth spurts has been associated with osteosarcoma development. Occupational factors such as job type, industry, and exposures to chemicals such as herbicides and chlorophenols have been suggested as risk factors for sarcomas. A case-control study found a significant increase in soft tissue sarcoma risk among gardeners (adjusted OR 4.1, 95% CI 1.00, 14.00), but not among those strictly involved in farming. A European-based study reported an increased risk in bone tumors among blacksmiths, toolmakers, or machine-tool operators (adjusted OR 2.14, 95% CI 1.08, 4.26). Maternal and paternal characteristics such as occupation, age, smoking status, and health conditions experienced during pregnancy also have been suggested as sarcoma risk factors and would be important to assess in future studies. The limited studies we identified demonstrate significant relationships with sarcoma risk, but many of these results now require further validation on larger populations. Furthermore, little is known about the biologic mechanisms behind each epidemiologic association assessed in the literature. Future molecular epidemiology studies may increase our understanding of the genetic versus environmental contributions to tumorigenesis in this often deadly cancer in children and adults.

• #2 The Epidemiology of Sarcoma | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/2045-3329-2-14

  Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. […] A recent meta-analysis reported that children with a history of hernias have a greater risk of developing Ewings sarcoma (adjusted OR 3.2, 95% CI 1.9, 5.7). […] The limited studies we identified demonstrate significant relationships with sarcoma risk, but many of these results now require further validation on larger populations.

• #3 Key Statistics for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/about/key-statistics.html

  The American Cancer Society’s estimates for soft tissue sarcomas in the United States for 2025 are: About 13,520 new soft tissue sarcomas will be diagnosed (7,600 in males and 5920 in females). About 5,420 people (2,960 males and 2,450 females) are expected to die of soft tissue sarcomas. […] These statistics include both adults and children. […] Certain types occur more often in certain parts of the body more often than others. For example, leiomyosarcomas are the most common type of sarcoma found in the abdomen (belly), while liposarcomas and undifferentiated pleomorphic sarcomas are most common in legs. But pathologists (doctors who specialize in diagnosing cancers by how they look under the microscope), may not always agree on the exact type of sarcoma. Sarcomas of uncertain type are very common.

• #4 Soft Tissue Cancer — Cancer Stat Facts

  https://seer.cancer.gov/statfacts/html/soft.html

  Estimated New Cases in 2025 13,520 […] Estimated Deaths in 2025 5,410 […] Soft tissue cancer represents 0.7% of all new cancer cases in the U.S. […] In 2025, it is estimated that there will be 13,520 new cases of soft tissue cancer and an estimated 5,410 people will die of this disease. […] The rate of new cases of soft tissue cancer was 3.5 per 100,000 men and women per year based on 20182022 cases, age-adjusted. […] For soft tissue cancer, death rates increase with age. […] The death rate was 1.3 per 100,000 men and women per year based on 20192023 deaths, age-adjusted. […] The percent of soft tissue cancer deaths is highest among people aged 6574. […] Using statistical models for analysis, age-adjusted rates for new soft tissue cancer cases have not changed significantly over 20132022. Age-adjusted death rates have been stable over 20142023.

• #5 Sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Sarcoma

  Sarcomas are rare cancers. […] The risk of a previously healthy person receiving a new diagnosis of bone cancer is less than 0.001%, while the risk of receiving a new diagnosis of soft-tissue sarcoma is between 0.0014 and 0.005%. […] The American Cancer Society estimates that in the United States there will be 3,970 new cases of bone sarcoma in 2023, and 13,400 new cases of soft-tissue sarcoma. […] Considering that the total estimated number of new cancer diagnoses (all types of cancer) is 1,958,310, this means bone sarcomas represent only 0.2% of all new cancer diagnoses (making them the 30th most common type of cancer) and soft-tissue sarcomas represent only 0.7% (making them the 22nd most common type of cancer) of all new cancer diagnoses in the US in 2023. […] These estimates are similar to previously reported data. […] Sarcomas affect people of all ages. Around 50% of bone sarcomas and 20% of soft-tissue sarcomas are diagnosed in people under the age of 35.

• #6

  https://www.populationmedicine.eu/Epidemiology-quality-of-care-and-costs-of-a-soft-tissue-sarcoma-cohort-results-from,165187,0,2.html

  Epidemiology, quality of care, and costs of a soft tissue sarcoma cohort: results from a high-resolution regional registry […] Soft tissue sarcomas (STS) are rare malignancies. Their low incidence and the complexity of their management demand their treatment at high-volume multidisciplinary centers to ensure an adequate quality of care. This study aimed to assess the epidemiology, quality of care, and costs of care for cases of STS in the Veneto Region. […] The incidence of soft tissue sarcoma was generally higher in males than in females. The temporal trends were flat for women, and rose slightly trend for men (APC = 0.9, p-value = 0.001). […] The availability of a high-resolution registry enabled a population-based epidemiological investigations on the direct costs of illness for patients with STS, affording an overview of the quality of their clinical care pathways.

• #7 Descriptive analysis of incidence, demographic characteristics, and survival outcomes of soft-tissue sarcoma of the extremities in Saudi Arabia | Scientific Reports

  https://www.nature.com/articles/s41598-024-79716-1

  Soft-tissue sarcomas are uncommon, aggressive, and histologically heterogeneous, malignant tumors. This study aimed to assess the prevalence and incidence of soft-tissue sarcomas of the extremities to facilitate early diagnosis and intervention in the Kingdom of Saudi Arabia. The incidence of sarcoma ranged from 2 to 2.9 cases per one million (average, 2.4 cases per one million). The incidence of soft-tissue sarcomas in the Kingdom of Saudi Arabia were lower than those in Western countries. The average incidence of soft-tissue sarcomas in the KSA was 2.4 cases per one million (range, 22.9 cases per one million). These incidence were notably lower than those reported in the United States (6.3 cases per 100,000 persons per year) and Europe (4.0 cases per 100,000 persons per year). The age distribution in our study revealed that soft-tissue sarcomas was more prevalent among younger age groups, with 22% of the cases occurring in the 19-30 years age group. Most cases occurred in the central region, followed by those in the Western and eastern regions (32%, 25%, and 21%, respectively). The most common soft-tissue sarcoma subtypes were liposarcoma (20.5%), malignant fibrous histiocytoma (13.2%), synovial sarcoma (11.1%), fibrosarcoma (9.9%), and leiomyosarcoma (8.2%). The survival rates identified in our study highlight the need for continued research and targeted interventions to improve outcomes for patients with soft-tissue sarcoma, particularly for those with more aggressive subtypes such as sarcoma NOS and rhabdomyosarcoma.

• #8 Sarcoma Surveillance: A Review of Current Evidence and Guidelines – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31651565/

  After initial treatment of sarcoma, disease progression may occur in the form of local recurrence, pulmonary metastases, or extrapulmonary metastases. As such, surveillance is an important aspect of management, but no universally accepted practice standards are found. […] In general, they agree that follow-up should be more intense in the early years after treatment, especially for high-grade sarcomas, and continue for at least 10 years. For local recurrence, data suggest that physical examination is usually sufficient for monitoring; in addition, some guidelines endorse imaging routinely, whereas others only as clinically indicated. For pulmonary metastasis, either radiograph or CT is recommended, with the latter having theoretical advantages but no proven survival benefit to date. Extrapulmonary metastases are rare in most sarcoma types, so the literature only supports extrapulmonary surveillance for certain diagnoses. […] Therefore, it is critical for providers to understand the existing research and guidelines to determine optimal surveillance strategies for their patients.

• #9

  https://journals.lww.com/jaaos/Fulltext/2020/02150/Sarcoma_Surveillance__A_Review_of_Current_Evidence.2.aspx?generateEpub=Article%7Cjaaos:2020:02150:00002%7C10.5435/jaaos-d-19-00002%7C

  After initial treatment of sarcoma, disease progression may occur in the form of local recurrence, pulmonary metastases, or extrapulmonary metastases. […] surveillance is an important aspect of management, but no universally accepted practice standards are found. […] existing guidelines contain flexibility in terms of both the frequency and modality of surveillance. […] they agree that follow-up should be more intense in the early years after treatment, especially for high-grade sarcomas, and continue for at least 10 years. […] For local recurrence, data suggest that physical examination is usually sufficient for monitoring; in addition, some guidelines endorse imaging routinely, whereas others only as clinically indicated. […] For pulmonary metastasis, either radiograph or CT is recommended, with the latter having theoretical advantages but no proven survival benefit to date. […] Extrapulmonary metastases are rare in most sarcoma types, so the literature only supports extrapulmonary surveillance for certain diagnoses. […] it is critical for providers to understand the existing research and guidelines to determine optimal surveillance strategies for their patients.

• #10 Patterns of Extrapulmonary Metastases in Sarcoma Surveillance

  https://www.mdpi.com/2072-6694/13/18/4669

  Soft tissue sarcomas (STS) most commonly metastasize to the lungs. Current surveillance guidelines variably recommend abdominal and pelvic imaging, but there is little evidence to support this. We sought to determine the proportion of initial pulmonary versus extrapulmonary metastases, the time to development of each, and factors to identify patients that would benefit from abdominopelvic surveillance. We retrospectively reviewed 382 patients who underwent surgical treatment for STS at a single institution. Of the 33% (126/382) of patients who developed metastases, 72% (90/126) were pulmonary, 22% (28/126) were extrapulmonary, and 6% (8/126) developed both simultaneously. Initial extrapulmonary metastases occurred later (log rank p = 0.049), with median 11 months (IQR, 5 to 19) until pulmonary disease and 22 months (IQR, 6 to 45) until extrapulmonary disease. Pulmonary metastases were more common in patients with high grade tumors (p = 0.0201) and larger tumors (p < 0.0001). Our multivariate analysis did not identify any factors associated with initial extrapulmonary metastases. A substantial minority of initial metastases were extrapulmonary; these occurred later and over a broader time range than initial pulmonary metastases. Moreover, extrapulmonary metastases are more difficult to predict than pulmonary metastases, adding to the challenge of creating targeted surveillance protocols.

• #11 Post-operative surveillance in soft tissue sarcoma: using tumorspecific recurrence patterns to direct approach – Zaidi – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/20903/html

  Significant challenges exist in creating surveillance recommendations for soft tissue sarcomas (STS) given the vast heterogeneity of recurrence patterns between histologic subtypes. […] Although surveillance protocols have been developed in parallel with our growing knowledge of cancer biology, many of these surveillance programs lack evidence for their effectiveness in detecting local or distant recurrences at a treatable stage. This is especially true in soft tissue sarcoma (STS), where the complex heterogeneity of the disease presents particular challenges in crafting effective surveillance strategies. […] The natural history of STS is determined largely by the histologic subtype of tumor and the anatomic site of origin. […] To this end, national and international groups have recommended surveillance strategies that encompass the diverse tumor behaviors that STS subtypes may exhibit.

• #12 The impact and efficacy of surveillance in patients with sarcoma of the extremities | Bone & Joint

  https://boneandjoint.org.uk/Article/10.1302/2046-3758.64.BJR-2016-0253.R1

  In this cross sectional study, the impact and the efficacy of a surveillance programme for sarcomas of the extremities was analysed. […] The surveillance programme accounts for 14% of all review appointments. […] Surveillance of sarcoma patients makes up a substantial amount of the workload of a sarcoma unit. The chance of cure following identification of local recurrence or metastatic disease, however, is small. Alternative methods of surveillance that allow better evaluation of the patients needs are recommended. […] A substantial amount of appointments in a sarcoma unit are for surveillance following curative treatment. […] Although chest radiographs are good in identifying metastatic disease, local recurrence is usually identified by the patient. […] The chance of cure following the identification of local recurrence or metastatic disease is small.

• #13 The Surveillance After Extremity Tumor Surgery (SAFETY) trial: protocol for a pilot study to determine the feasibility of a multi-centre randomised controlled trial | BMJ Open

  https://bmjopen.bmj.com/content/9/9/e029054

  Post-treatment STS surveillance is an integral element of patient care. […] In order to assess the available evidence, we completed a systematic review of the available randomised controlled trial (RCT) evidence for surveillance in sarcoma management. […] Regular, intensive surveillance is more likely to identify recurrent disease earlier than would less intensive surveillance. […] The costs that healthcare systems incur as a result of sarcoma surveillance are substantial and could be in excess of US$20,000 for high-grade sarcomas. […] The ultimate goal of the SAFETY trial is to provide high-quality evidence for surveillance strategies following the treatment of STS, which will allow for the development of evidence-based clinical practice guidelines for patients with sarcoma worldwide.

• #13 The Surveillance After Extremity Tumor Surgery (SAFETY) trial: protocol for a pilot study to determine the feasibility of a multi-centre randomised controlled trial | BMJ Open

  https://bmjopen.bmj.com/content/9/9/e029054

  Following the treatment of patients with soft tissue sarcomas (STS) that are not metastatic at presentation, the high risk for local and systemic disease recurrence necessitates post-treatment surveillance. […] The most appropriate surveillance frequency and modality remain unknown and, as such, clinical practice is highly varied. […] Routine follow-up after completing sarcoma treatment is standard practice in the first 5 years after surgery. […] Surveillance strategies for long-term follow-up of patients with sarcoma have not been well researched and current guidelines are based on expert opinion, not on high-quality evidence. […] The current National Comprehensive Cancer Network (NCCN) guidelines suggest that stage II or III tumours should be followed with chest imaging (CT or CXR) every 2-6 months for the first 2-3 years and then annually thereafter, while stage I tumours could be followed less frequently during the first 2-3 years.

• #14 University of California Health Sarcoma Trial → Surveillance AFter Extremity Tumor surgerY

  https://clinicaltrials.ucbraid.org/trial/NCT03944798

  Following treatment for a primary extremity sarcoma, patients remain at risk for the development of local and systemic disease recurrence. […] Metastasis (distant recurrence) to the lung is the most frequent single location of disease recurrence in sarcoma patients, occurring in almost half of all patients. Therefore, careful post-operative surveillance is an integral element of patient care. […] Surveillance strategies have not been well researched and have been identified as the top research priority in the extremity sarcoma field. […] Post-treatment STS surveillance is an integral element of patient care. […] The orthopaedic oncology field has identified sarcoma surveillance strategy as the top research priority in the field. […] In order to fill the evidence gap in sarcoma surveillance, a large international RCT is required. […] Ultimately, the SAFETY trial will provide the necessary evidence to develop evidence-based surveillance guidelines, and is poised to have a significant impact on the post-operative care and outcomes of extremity STS patients.

• #15 MRI Surveillance After Soft-Tissue Sarcoma Surgery Detects Local Recurrence | Applied Radiation Oncology

  https://www.appliedradiationoncology.com/articles/mri-surveillance-after-soft-tissue-sarcoma-surgery-detects-local-recurrence

  MRI-based surveillance after surgical treatment of musculoskeletal soft-tissue sarcomas (STS) shows utility for detecting clinically occult local recurrences (LRs), potentially improving patient outcomes, according to a study published in the American Journal of Roentgenology (AJR). […] When used at a high intensity, MRI-based surveillance can detect many clinically occult LRs, although the studies are small, occasionally conflicting, and often of poor quality, wrote corresponding author Natalia Gorelik, MD, from the department of diagnostic radiology at McGill University Health Center in Montreal, Quebec, Canada. […] A survival benefit could be associated with imaging use, but further research is needed to evaluate the causality of any observed survival differences. […] Ultimately, when MRI-based surveillance of musculoskeletal STSs was used at a high intensity, 53% (95% CI, 3671%) of LRs were discovered with MRI.

• #16 MRI Surveillance After Soft-Tissue Sarcoma Surgery Detects Local Recurrence | Applied Radiation Oncology

  https://www.appliedradiationoncology.com/articles/mri-surveillance-after-soft-tissue-sarcoma-surgery-detects-local-recurrence

  Four studies reported trends toward better survival for imaging-detected LRs or with more frequent imaging use. […] Although definite conclusions on the value of MRI-based surveillance are challenging to draw, this study provides a comprehensive overview of the available evidence on the topic, the authors wrote.

• #17

  https://www.healio.com/news/hematology-oncology/20180216/tumor-subtype-location-should-guide-mri-surveillance-after-soft-tissue-sarcoma-excision

  Routine MRI surveillance for local recurrence may translate into better oncologic outcome in extremity soft tissue sarcoma. […] All commenters acknowledged the lack of consensus recommendations due to the challenges of creating well-designed studies for sarcomas, a rare and heterogeneous group of malignancies. […] In summary, use of periodic MRI surveillance for soft tissue sarcoma local recurrence should be guided by a case-by-case analysis based on relevant clinical data, tumor location and sarcoma subtype. […] It is important for providers to remain cognizant of the statistically low chance of discovering an asymptomatic local recurrence, as well as the possibility of false positives that may lead to unnecessary procedures.

• #18 Chest CT Imaging Patterns Contradict ACR Guidelines for Extremity Sarcoma Staging and Surveillance

  https://www.onclive.com/view/chest-ct-imaging-patterns-contradict-acr-guidelines-for-extremity-sarcoma-staging-and-surveillance

  According to findings from a retrospective review published in the Journal of Surgical Oncology investigators at the University of California (UC) Davis in Sacramento evaluated their own compliance with the American College of Radiology (ACR) guidelines for sarcoma staging and surveillance. The guidelines, which have been published since 2015, recommend pulmonary staging and surveillance with chest CT without contrast for patients with extremity sarcoma. […] The results showed that more patients received CT with contrast both before and after the publication of the guidelines. Moreover, 79.2% of patients final surveillance CT scans after 2015 were performed with contrast, adding $297,704 worth of additional patient and institutional expenses. […] We realized that from an institutional standpoint, this was an additional cost of about $300,000 to the health system for [ordering CTs] with contrast when the ACR declared that it was not advantageous for these patients.

• #19 Sarcoma surveillance: the patient experience and impact on QoL – Health Research Authority

  https://www.hra.nhs.uk/planning-and-improving-research/application-summaries/research-summaries/sarcoma-surveillance-the-patient-experience-and-impact-on-qol/

  Retroperitoneal (back of the abdomen), abdominal, and pelvic sarcomas (cancers of connective tissue) are malignant tumours. Sarcomas are generally treated with surgery alone, followed by a follow-up plan that includes regular clinic visits and scans (surveillance) to check the sarcoma has not grown back or spread to other parts of the body. The impact of surveillance and surveillance frequency on quality of life and long-term survival is unknown. […] Surveillance can cause significant anxiety, termed scanxiety, and disruption of daily life for patients. […] The interviews will explore patients lived experience of sarcoma surveillance and patients quality of life related to surveillance, the effect of high frequency compared to lower frequency surveillance on patients quality of life, and patients acceptance with regards to different radiological surveillance frequencies. In addition, clinicians attitudes towards sarcoma surveillance and their views with regards to how sarcoma surveillance impacts on patients quality of life will be explored. […] This information will help clinicians and policy makers to make meaningful changes to the surveillance strategy to potentially improve patients quality of life and the quality of care patients receive and reduce anxiety and distress suffered by patients.

• #20 Preventative medicine and surveillance in sarcoma survivorship

  https://researchfeatures.com/preventative-medicine-surveillance-sarcoma-survivorship/

  Surviving sarcoma is hard in and of itself, but survivors often experience long-term effects in the years after their treatment has finished. […] However, sarcoma survivors often do not receive adequate surveillance and follow-up care. […] Due to the long-term effects of sarcoma survivorship, good post-treatment care is key to ensuring good health and quality of life. […] For this reason, surveillance of these patients for risk factors of chronic conditions is important, as well as practising preventative medicine and encouraging a healthy lifestyle to reduce the risk of them occurring. […] The risk factors for coronary artery disease in sarcoma survivors greatly exceed those in the general population. […] At the Sarcoma Survivorship Clinic, Professor Baker and colleagues have provided comprehensive, holistic care to help manage late effects of cancer treatment and prevent the onset of conditions such as CAD.

• #21 Surveillance After Extremity Tumor Surgery International Randomized Controlled Trial | University of Iowa Clinical Research and Trials

  https://clinicaltrials.uihealthcare.org/studies/surveillance-after-extremity-tumor-surgery-international-randomized-controlled-trial

  Post-treatment STS surveillance is an integral element of patient care. […] The orthopaedic oncology field has identified sarcoma surveillance strategy as the top research priority in the field. […] In order to fill the evidence gap in sarcoma surveillance, a large international RCT is required. […] Ultimately, the SAFETY trial will provide the necessary evidence to develop evidence-based surveillance guidelines, and is poised to have a significant impact on the post-operative care and outcomes of extremity STS patients.

• #22 Chest X-Rays or CT scans? An international trial to evaluate the best follow-up for soft tissue sarcoma (SAFETY trial) | Sarcoma UK

  https://sarcoma.org.uk/research/chest-x-rays-or-ct-scans-an-international-trial-to-evaluate-the-best-follow-up-for-soft-tissue-sarcoma-safety-trial/

  Surgery and radiotherapy are the key treatments for most soft tissue sarcomas. […] But surveillance strategies have not been well-researched. This means current guidelines are based on expert opinion, rather than evidence. […] Earlier detection of a sarcoma coming back or spreading may improve survival, but to date no study has definitively proved this. This trial will provide the evidence to ensure that patient care and follow-up is as effective as it can be, based on high quality evidence.

• #23 Surveillance Post Surgery for Retroperitoneal Soft Tissue Sarcoma

  https://www.mdpi.com/1718-7729/30/3/211

  Risk stratification tools exist and are available for use in routine practice. Their use will likely support more individualised post-operative surveillance moving forward. […] Surveillance will likely be underpinned by serial radiological imaging for the medium term. […] However, developments in genomics offer hope for biomarkers such as ctDNA to impact patient care positively in the future and further support individualised patient care pathways.

• #24 Sarcoma Journals, Videos & Insight | Touch Oncology

  https://touchoncology.com/speciality/sarcoma/

  Sarcomas are a heterogeneous group of mesenchymal malignancies, with more than 50 subtypes identified. […] Sarcomas are a diverse group of rare muscle, bone and connective tissue tumours. Within soft-tissue sarcoma alone, there are over 70 subtypes, which demonstrate substantial heterogeneity in biology, genetics and clinical behaviour. […] This review serves to discuss the epidemiology, molecular biology, clinical behavior, and the current treatment modalities of TGCT that […] Soft tissue sarcomas represent a group of over 80 rare malignant tumors that arise from tissues of mesenchymal origin throughout the body.

• #25 Epidemiology, incidence, and survival of synovial sarcoma of children: a SEER database analysis – Zhu – Translational Pediatrics

  https://tp.amegroups.org/article/view/126976/html

  Roughly 5% to 10% of soft tissue sarcomas fall under the category of synovial sarcomas (SSs), a rare and malignant tumor originating from soft tissues with unclear differentiation, primarily affecting teenagers and young adults. […] The goal of this study was to assess the latest survival rates for SS of children and the risk factors affecting survival using the Surveillance, Epidemiology and End Results (SEER) database. […] A total of 130 patients were enrolled in the study. In the overall survival analysis, age (P=0.01), male (P=0.04), no surgery (P0.01), chemotherapy (P0.01), primary tumor site in soft tissue (P=0.02), and in distant of SEER stage (P0.01) were associated with a worse prognosis in children with SS. […] Multivariate analysis showed that chemotherapy and in distant of SEER stage were independent indicators of unfavorable prognosis.

• #26 Epidemiology, incidence, and survival of synovial sarcoma of children: a SEER database analysis – Zhu – Translational Pediatrics

  https://tp.amegroups.org/article/view/126976/html

  The study adhered strictly to the principles outlined in the Declaration of Helsinki (as revised in 2013). […] The findings of this investigation were quite significant. […] A bad prognosis was linked to having male partners. […] Additionally, it was shown that initial soft tissue tumors had a better prognosis. […] It is perplexing, nevertheless, that children who receive chemotherapy or surgery have a worse prognosis. […] The regional or remote spread of SS is known to be connected to poor prognosis, similar to many soft tissue sarcomas. […] Male sex has been linked to a worse prognosis in SS, according to earlier reports. […] One of the most significant prognostic factors in SS is age, as is well recognized. […] The lowest overall survival rate was observed to be among children under 5 years old.

• #27 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/277297-overview

  Angiosarcomas are rare neoplasms. Approximately 50% of angiosarcomas occur in the head and neck, but they account for less than 0.1% of head and neck malignancies. […] Around 2% of soft tissue sarcomas in general and 4% of cutaneous soft tissue sarcomas are angiosarcomas. […] A study of the Surveillance, Epidemiology, and End Results database found that the incidence of all soft tissue sarcomas from 1973 to 2006 was 5.9 per 100,000 persons. […] This would yield an incidence of approximately 1.2 per million for angiosarcomas. […] Worldwide incidence is also low; in the United Kingdom, for example, the National Cancer Intelligence Network (NCIN) reported that angiosarcomas represent 3.3% of all soft tissue sarcomas, with an incidence rate of 1.5 per million.

• #28 Breast sarcoma: Epidemiology, risk factors, clinical presentation, diagnosis, and staging – UpToDate

  https://www.uptodate.com/contents/breast-sarcoma-epidemiology-risk-factors-clinical-presentation-diagnosis-and-staging

  Breast sarcomas are rare; they account for less than 1 percent of all breast malignancies and less than 5 percent of all soft tissue sarcomas. In data compiled from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute (NCI), the annual incidence of breast sarcomas was 4.6 cases per million women. […] The specific incidence of secondary, therapy-related breast sarcomas is difficult to discern. Secondary or treatment-related breast sarcomas most frequently arise following breast cancer treatment with radiation therapy (RT). However, the absolute magnitude of the risk of a secondary breast sarcoma in women who undergo RT for breast cancer appears to be small. […] Angiosarcoma is the specific sarcoma type that is associated most strongly with treatment for a previous breast cancer. In a population-based study in Los Angeles County, the adjusted relative risk of developing an angiosarcoma for women with a prior diagnosis of breast cancer compared with women without breast cancer was 59. Furthermore, women who receive RT as a component of breast cancer treatment have a 9- to 16-fold increase in the relative risk of developing an angiosarcoma relative to those treated with other modalities. However, despite these high relative risks, the absolute magnitude of risk for a treatment-related angiosarcoma after RT remains small.

• #29 Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China | Cancer Biology & Medicine

  https://www.cancerbiomed.org/content/16/3/565

  Objective: Soft tissue sarcomas (STSs) are rare malignancies deriving from mesenchyme. In this study, we reported the epidemiology of STS in China using population-based cancer registry data. […] In 2017, qualified data from 339 cancer registries were included in the national database. All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly. […] Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence. […] The burden of STS is not serious in China relatively. However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.

• #30 Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China | Cancer Biology & Medicine

  https://www.cancerbiomed.org/content/16/3/565

  With worlds largest population, 23.7% of the global new cancer cases were estimated to occur in China in 2018. However, little is known about the incidence of STS in this country. […] In this study, we described the distribution of histological subtypes and primary sites of STS using updated data from 339 population-based cancer registries. Estimation of the incidence of STS in the whole Chinese population was made as well and was reported for GIST and STSs other than GIST (non-GIST STSs) separately. […] This study is the first in China mainland to report the nationwide incidence of STS as well as the distribution of histological subtypes and primary sites.

• #31

  https://ijph.tums.ac.ir/index.php/ijph/article/view/31445

  We aimed to investigate the patterns of incidence and prevalence of bone sarcoma (BS) and soft tissue sarcoma (STS), morphology as well as geographical distribution in the elderly in Iran. […] The annual crude incidence rates of sarcomas in males (0.80 per 100,000) were more than in females (0.55 per 100,000) in all years. The total combined crude incidence in 2014 years was obtained at 0.67 per 100,000 people. […] Even though such sarcoma is more prevalent in elderly men, its incidence was also observed in lower-aged female groups. In addition, the incidence rate of BS was lower in comparison with that of STS, and the patients often exhibited an unknown degree of sarcoma.

• #32 Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0246958

  Since 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France. […] The nationwide incidence of sarcoma or TIM (2013-2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. […] Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/106/year, higher than previously reported. […] This nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (1106/year) are less likely to be included in clinical trials.

• #33 Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0246958

  The global nationwide incidence of sarcoma has seldom been reported. Taking advantage of the organized reference center networks and expert pathology reviewing, we report here the incidence of the different histological subtypes of sarcomas and TIM from the NETSARC+ database from 2013 to 2016. […] The official numbers of the French population are 65.56, 66.13, 66.42 and 66.60 million inhabitants in respectively 2013, 2014, 2015, 2016. The estimated incidences of sarcomas and tumors of intermediate malignancy from 2013 to 2016 were 89.05, 93.04, 100.18, and 98.00 per million inhabitants respectively. Over these 4 years, the estimated yearly incidence of sarcomas and TIM was therefore 95,1/106/year. […] The present work also confirms that sarcoma and TIM are a highly fragmented group of diseases. Over 150 different histotypes or groups of histotypes relevant for clinical practice are listed. […] The individual incidence of different sarcoma histotypes ranged from 10/106 to less than 0.01/106, i.e. a 1000-fold difference in incidence. […] Overall, this calls for a revision of the criteria to define standard treatment for such rare tumors where phase III are hardly or not feasible.

• #34 The Epidemiology of Sarcoma | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/2045-3329-2-14

  Future molecular epidemiology studies may increase our understanding of the genetic versus environmental contributions to tumorigenesis in this often deadly cancer in children and adults. […] The purpose of this review is to perform a thorough evaluation of the existing literature on the epidemiology and etiology of sarcomas. […] The majority of the assessed exposures lacked enough evidence needed to draw strong conclusions, because these exposures have not been adequately studied. More frequently studied exposures, which were found to be significantly associated with sarcoma occurrence in the majority of circumstances, suffered from the occasional inconsistent result. […] Ultimately, drawing strong conclusions can be difficult to make because many of results from these studies have not been adequately replicated. […] The available literature and research on sarcoma risk has shown that these rare diseases are difficult to study.

• #35 Patterns of Extrapulmonary Metastases in Sarcoma Surveillance

  https://www.mdpi.com/2072-6694/13/18/4669

  Of the 33% of patients with primary STS that developed metastatic disease after resection, 22% were initially diagnosed with extrapulmonary metastases, and an additional 6% with simultaneous pulmonary and extrapulmonary metastases. Initial extrapulmonary metastases occurred later and over a broader time range than pulmonary metastases. While patients with larger, higher grade tumors were more likely to develop pulmonary metastases, we were unable to identify patient or tumor variables that were associated with an increased risk of extrapulmonary metastases. These findings suggest that routine abdominal and pelvic imaging may detect new metastatic disease in a substantial minority of patients. Future studies with larger numbers may identify risk factors for extrapulmonary disease, but at this time we are unable to predict which, if any, patients are more or less likely to benefit from routine abdominopelvic imaging. Further research is needed to evaluate the potential advantages of early detection of extrapulmonary metastases on treatment and survival. Additionally, better understanding the psychosocial impact of increased imaging, the potential morbidity of investigating false positive findings, and the overall cost-effectiveness of advanced imaging will be necessary to develop definitive surveillance recommendations.

• #36 The Epidemiology of Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3564705/

  The purpose of this review is to perform a thorough evaluation of the existing literature on the epidemiology and etiology of sarcomas. Thus, conclusions will be made on the risk factors that are established, which will be of benefit on drawing conclusions on appropriate preventative guidelines. […] The available literature and research on sarcoma risk has shown that these rare diseases are difficult to study. The challenge of studying a rare outcome is that it often requires the assessment of rare exposures. Implementing studies with these characteristics often results in the creation of null or conflicting results. For example, the relationship between occupational factors and the risk of sarcoma development appears to be one of the more common and frequently studied topics. However, even with the plethora of papers published on this particular subject matter, clear conclusions can be difficult to draw. Many of the occupational exposure studies lacked the statistical power and adequate sample sizes needed in order to create strong conclusions, thus again, we only can conclude that the identified associations are suggestive. Furthermore, many of these studies were required to group sarcoma subtypes together in order to gain enough statistical power to fully implement their study, which can result in the assumption that many of the sarcoma subtypes have the same etiologic properties.

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