Materiały źródłowe

• #1 Ewing Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559183/

  Ewing sarcoma is the second most common primary bone malignancy in adolescents and young adults with a median age of 15 years and accounts for less than 5% of all soft tissue sarcomas. There are more than 200 cases per year in the United States. The incidence of Ewing sarcoma in the United States was 2.93 per million between 1973 and 2004. […] The peak incidence is between 10 and 15 years, with around 30% of the cases arising in children under 10 and another 30% in adults over 20. There is a male predominance with a male-to-female ratio of 3 to 1. Whites are much more frequently affected than Blacks, Asians, Hispanics, or Africans. This significant racial discordance has yet to be explored. The actual incidence of Ewing sarcoma in older populations is unknown.

• #2 Ewing Tumor Types Statistics | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/ewing-tumor/about/key-statistics.html

  Ewing tumors (Ewing sarcomas) are not common. About 1% of all childhood cancers are Ewing tumors. About 200 children and teens are diagnosed with Ewing tumors (sarcomas) in the United States each year. […] Most Ewing tumors occur in teens, but they can also affect younger children, as well as adults (mainly in their 20s and 30s). […] Slightly more males than females develop these cancers. These tumors are much more common among White people, either non-Hispanic or Hispanic. This disease is very rare among African Americans, and it also seldom occurs in other racial groups.

• #3 Ewing sarcoma: Statistics and more

  https://www.medicalnewstoday.com/articles/how-common-is-ewing-sarcoma

  Ewing sarcoma is the second most common bone cancer in children and younger adults. However, it is still very rare. […] Ewing tumors account for about 1% of all childhood cancers, affecting approximately one in a million individuals. […] Ewing sarcoma is a rare bone cancer that mainly affects children, adolescents, and young adults. […] An estimated 87% of Ewing sarcomas affect the bone. These sarcomas may also affect surrounding soft tissue. […] Ewing sarcoma accounts for 10-15% of all bone sarcomas and less than 5% of all soft tissue sarcomas. […] One-third of all Ewing tumors are present in the pelvis and spine. […] There are more than 200 cases of Ewing sarcoma diagnosed in children and teens in the United States each year. […] Between 1973 and 2004, the incidence of Ewing sarcoma in the U.S. was 2.93 per one million people.

• #4 Ewing Sarcoma – American Association for Cancer Research (AACR)

  https://www.aacr.org/patients-caregivers/cancer/ewing-sarcoma/

  Ewing sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. Ewing tumors often occur in teenagers and young adults. The median age of patients with Ewing sarcoma is 15 years. […] According to the National Cancer Institute, the incidence of Ewing sarcoma across people of all ages is approximately one case per million people per year. The incidence in people ages 10 to 19 is between 9 and 10 cases per million, and the same analysis suggests that the incidence of Ewing sarcoma in the United States is nine times greater in Caucasians than in African Americans.

• #5 Ewing sarcoma – Wikipedia

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

  Ewing sarcomas represent 16% of primary bone sarcomas. In the United States, they are most common in the second decade of life, with a rate of 0.3 cases per million in children under 3 years of age, and as high as 4.6 cases per million in adolescents aged 15-19 years. Nearly 80% of patients are aged less than 20 years of age. It is uncommon in patients younger than 5 years and older than 30 years. […] Internationally, the annual incidence rate averages less than 2 cases per million children. In the United Kingdom, an average of six children per year are diagnosed; mainly males in early stages of puberty. With occurrences primarily arising in older children and teenagers, one causal theory is puberty, e.g. its rapid growth spurts making bone tissue more cancer susceptible during development years. […] Ewing sarcoma occurs about 10- to 20-fold more commonly in people of European descent compared to people of African descent. Ewing sarcoma is the second most common bone cancer in children and adolescents, with poor prognosis and outcome in ~70% of initial diagnoses and 10-15% of relapses.

• #6 Ewing sarcoma | Bone Cancer Research Trust

  https://www.bcrt.org.uk/information/information-by-type/ewing-sarcoma/

  Ewing sarcoma is the second most common type of primary bone cancer found in young people. […] Each year in the UK around 90 people are diagnosed with Ewing sarcoma. […] Ewing sarcoma tends to be more common in White people than in Black and Asian people. […] Ewing sarcoma is more common in males than females. However, in people under 15 years old, males and females are affected equally. […] Around 2 in every 100 children with cancer have Ewing sarcoma. […] Treatments for Ewing sarcoma have not changed much over the past 30 years, which means survival rates have not improved. […] 60 out of every 100 people with Ewing sarcoma are alive 5 years after their diagnosis but some may still be receiving treatment. This number is lower if the cancer is metastatic (has spread) at diagnosis.

• #7 Incidence of Ewing’s Sarcoma in Different Age Groups, Their Associated Features, and Its Correlation With Primary Care Interval

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8054948/

  Ewings sarcoma is a rare small round cell bone tumor originating from neuroectoderm and undifferentiated neuroepithelial cells, having an annual incidence of approximately one case per million in the United States. […] The peak time for the occurrence of Ewings sarcoma is from 15 years to 20 years of age. […] Factors causing a prolonged primary care interval include early age of onset, non-specific clinical presentation, and insufficient knowledge of the primary care physician, which results in poor prognosis. […] The incidence of Ewings sarcoma is significantly higher in Caucasians as compared to Asians and Africans. […] The mean age for diagnosis is 14-15 years while increasing age at diagnosis leads to worse outcomes. […] The objective of this study is to analyze the age distribution along with major clinical features at the time of presentation including the site of involvement, distant metastasis, and regional lymphadenopathy. […] The primary care interval is long in Ewing’s sarcoma patients.

• #8

  https://www.orthobullets.com/pathology/8047/ewings-sarcoma

  Epidemiology […] Incidence […] 3/1,000,000 (rare) […] second most common primary malignant bone tumor in children […] accounts for 3% of all pediatric malignancies and 10% of all primary malignant bone tumors […] Demographics […] male:female ratio = 1.5:1 […] 5-25 years of age most common […] 80-90% of patients are 20 years of age with peak incidence between 10-15 years old […] uncommon in African Americans and Asian populations […] Location […] bone […] split evenly between axial skeleton and long bones of appendicular skeleton […] metadiaphysis of long bones (~50%) […] femur […] tibia […] humerus […] pelvis (~25%) […] scapula […] soft tissue […] rare […] […] […] 26-28% have macrometastases on presentation (lungs, bone, bone marrow)

• #9 Ewing Sarcoma: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/990378-overview

  The overall annual incidence of Ewing sarcoma is approximately 1 case per 1 million per year in the United States. The incidence from birth to age 20 years is 2.9 cases per million population. Approximately half of all patients are aged 10-20 years at the time of first diagnosis, making this the second most common primarily malignant bone tumor in children and adolescents. Cases have been reported from birth through 80 years, although very infrequently. […] The incidence of these tumors in Whites is at least 9 times higher than it is in Blacks. This finding contrasts with that observed in osteosarcoma, which has a relatively equal racial distribution. African countries report similar incidences, with a paucity of Ewing sarcoma. […] The incidence of Ewing sarcoma in females is 2.6 cases per million population, compared with 3.3 cases per million population in males. […] The incidence of these tumors peaks in the late teenage years. Overall, 27% of cases occur in the first decade of life, 64% of cases occur in the second decade, and 9% of cases occur in the third decade.

• #10 Ewing’s sarcoma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Ewing%27s_sarcoma_epidemiology_and_demographics

  Ewing’s sarcoma is the second most common malignant bone tumor in patients younger than 20 years. Ewing’s sarcoma typically occurs in children and adolescents between 10 and 20 years of age. The overall incidence of Ewing’s sarcoma is 0.1 cases for 100,000 individuals. The incidence of Ewing’s sarcoma in children aged 10 to 19 years is 1 case per 100,000 individuals. Males are more commonly affected with Ewing’s sarcoma than females. […] The incidence of Ewing’s sarcoma has remained unchanged for 30 years. The overall incidence of Ewing’s sarcoma is 0.1 cases per 100,000 individuals. The incidence of Ewing’s sarcoma in children aged 10 to 19 years is 1 case for 100,000 individuals. […] Incidence of Ewing’s sarcoma in the United States is nine times greater in whites than in African Americans, with an intermediate incidence in Asians. The relative paucity of Ewing’s sarcoma in people of African or Asian descent may be explained, in part, by a specific polymorphism. The polymorphism associated with the increased risk is found at a much higher frequency in whites than in blacks or Asians, possibly explaining the epidemiology of the relative infrequency of Ewing’s sarcoma in the latter populations. Ewing’s sarcoma usually affects individuals of the Caucasian race. African American individuals are less likely to develop chordoma.

• #11

  https://link.springer.com/article/10.1007/s12094-024-03602-5

  Ewing sarcoma is a small round-cell sarcoma characterized by gene fusion involving EWSR1 (or another TET family protein like FUS) and an ETS family transcription factor. The estimated incidence of this rare bone tumor, which occurs most frequently in adolescents and young adults, is 0.3 per 100,000/year. […] ES is the third most frequent malignant bone tumor in humans and the second most frequent after osteosarcoma in children. Median age at diagnosis is 15 years, and the male sex predominates (1.5:1). […] ES is practically non-existent in African and Afro-American populations, while the estimated incidence is 0.3 per 100,000/year in Caucasian adults. […] ES is a systemic disease. Although only 25% of patients have obvious metastases at diagnosis, in the past, 80-90% of patients with apparently localized disease died when treatment was reduced to a local approach. Disease dissemination is predominantly hematogenous, being lung, bones, and bone marrow as the most common metastatic sites.

• #12 Ewing sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/ewing-sarcoma?lang=us

  Ewing sarcoma typically occurs in children and adolescents between 10-20 years of age (95% between 4-25 years of age) and has a slight male predilection (M:F 1.5:1) 1,2. […] The Ewing sarcoma family of tumors primarily occurs in White patients. In the United States, the incidence in the Asian/Pacific Islander population is about one-half that in the White population, while the incidence in the Black population is one-ninth that in White population 12.

• #13 Ewings Sarcoma Market Size, Trends and Forecast 2024-2034

  https://www.imarcgroup.com/ewings-sarcoma-market

  The annual incidence of Ewing sarcoma is about one case per million people. […] The incidence from infancy to age 20 is 2.9 incidences per million people. […] Whites are at least nine times more likely than Blacks to develop these malignancies. […] The incidence of Ewing sarcoma in females is 2.6 cases per million population, compared to 3.3 occurrences per million population in males. […] These tumors are more common in late adolescence.

• #14 Ewing Sarcoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/ewing-sarcoma/

  Ewing sarcoma primarily affects children and adolescents. It is the third most common type of bone tumor and accounts for approximately 1% of all pediatric cancers, affecting approximately 200 children in the United States (US) each year. […] The incidence of Ewing sarcoma is highest among those in their teens, but the disease also can develop in children younger than age 10 years and in adults older than age 20 years. Ewing sarcoma most commonly affects patients who are White (Hispanic or non-Hispanic) and is rare in other races. It is more likely to affect males than females.

• #15 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  Dramatic improvements in survival have been achieved for children and adolescents with cancer. […] For Ewing sarcoma, the 5-year survival rate has increased from 59% to a range of 80% to 85% for children younger than 15 years and from 20% to 69% for adolescents aged 15 to 19 years. […] In the United States between 2016 and 2020, the National Childhood Cancer Registry (NCCR) reported an incidence rate of Ewing sarcoma and related sarcomas of bone of 3.0 cases per 1 million in children and adolescents younger than 20 years. […] The incidence of Ewing sarcoma in the United States is nine times greater in White people than in Black people, with an intermediate incidence in Asian people. […] Based on data from 1,426 patients entered on European Intergroup Cooperative Ewing Sarcoma Studies, 59% of patients are male and 41% are female.

• #16

  https://link.springer.com/article/10.1007/s10552-023-01737-4

  The incidence of Ewing sarcoma varies according to race and ethnicity, and genetic susceptibility is known to affect disease risk. […] Ewing sarcoma has several well-known epidemiologic characteristics. Most notably, it is extremely rare in individuals of African descent and also has far lower incidence in Asian than in non-Hispanic White individuals. […] Our study confirms and quantifies this at about a 14-fold risk difference. We also observed that Asian and Hispanic individuals also have a significantly decreased risk. […] While a lower incidence of Ewing sarcoma in Asia was noted as early as 1980 in China, our results quantify this risk difference within a single population (i.e., the birth cohort of California) as nearly one-half. […] Besides race and ethnicity, we note a significantly increased risk of Ewing sarcoma in those with a heavier birthweight, which was not identified by prior analyses to our knowledge.

• #17 Ewing Sarcoma | Oncohema Key

  https://oncohemakey.com/ewing-sarcoma/

  Ewing sarcoma is the second most common primary bone sarcoma in children and young adults, following osteosarcoma. The annual incidence of Ewing sarcoma in the population younger than 20 years is approximately 2.9 per million. It is slightly more common in males (1.2:1 male:female). […] Risk factors for Ewing sarcoma remain largely obscure. The disease is not thought to be familial and only rarely occurs as a second malignancy. Epidemiologic studies have suggested associations between a history of hernia and paternal occupation and risk of developing Ewing sarcoma. The most unusual feature of the epidemiology of Ewing sarcoma is its predilection for Caucasians, in whom it is more frequent than among Asians, and 6 times more frequent than among Africans. […] This epidemiology is reproduced in sub-Saharan Africa, where rates of Ewing sarcoma are similar to those seen in North American people of African ancestry. This pattern suggests a genetic component to the disease. […] The genetic epidemiology of Ewing sarcoma has been investigated further by genome-wide associations, with three risk loci being identified.

• #18

  https://journals.lww.com/jpho-online/fulltext/2008/06000/changes_in_incidence_and_survival_of_ewing_sarcoma.3.aspx

  The US population-based cancer registry Surveillance, Epidemiology, and End Results (SEER) database provides an opportunity to evaluate the incidence and survival rates of Ewing sarcoma (ES) for the past 3 decades. […] The overall incidence of ES seemed to remain unchanged with an average of 2.93 cases/1,000,000 reported annually between 1973 and 2004. […] The incidence of ES has not increased appreciably over the last 30 years. A marked decrease in the proportion of unstaged cases may be reflective of diagnostic improvement or changes in reporting. There is a clear improvement in survival for both localized and metastatic disease.

• #19 SciELO Brazil – Survival outcome among patients with Ewing’s sarcoma of bones and joints: a population-based cohort study Survival outcome among patients with Ewing’s sarcoma of bones and joints: a population-based cohort study

  https://www.scielo.br/j/spmj/a/swmh5gKGCYhxbKrcR5VGxpD/?lang=en

  The aim here was to elucidate the current survival condition of patients diagnosed with Ewings sarcoma of the bones and joints and determine independent risk factors associated with the prognosis. […] Retrospective cohort study based on the Surveillance, Epidemiology and End Results (SEER) database in the United States. […] In the United States, the overall incidence rate of Ewings sarcoma is approximately 0.1 case per 100,000 individuals per year, and this rate had not undergone any obvious change over past 30 years. An estimated 90% of these patients are under 20 years old, and the death rate is approximately 0.05 cases per 100,000 individuals per year. Additionally, most cases of Ewings sarcoma of the bones and joints are found in the limbs, pelvis or spine. […] The SEER Program is supported by the National Cancer Institute of the United States and has provided statistical information on tumor cases since 1973. It collects data on cases diagnosed with cancer throughout the United States, with an estimated 28% of the United States population covered. The SEER registry is a validated database that is frequently applied for cancer survival studies.

• #20 Ewing sarcoma (brief information)

  https://www.gpoh.de/kinderkrebsinfo/content/diseases/solid_tumours/ewing_sarcoma/pohpatinfoewingkurz20101215/index_eng.html

  Ewing sarcomas are the second most common primary bone tumours in childhood and adolescence; they account for about 2 % of all pediatric malignancies. According to the German Childhood Cancer Registry (Mainz, Germany), approximately 50 children and adolescents under the age of 18 (about 3 of 1,000,000) are diagnosed with Ewing sarcoma in Germany per year. […] Ewing sarcoma may develop at any age; however, the frequency of this type of cancer peaks in the second decade of life. In childhood and adolescence (age group 0-17 years), the median age at diagnosis is 13 years, with adolescents between 12 and 17 years of age being the most frequently affected. Nevertheless, Ewing sarcomas are also observed in babies, infants, and school children. Overall, boys and male adolescents are more frequently affected by the disease than girls (gender ratio: 1.3:1).

• #21 Ewing Sarcoma as Secondary Malignant Neoplasm—Epidemiological and Clinical Analysis of an International Trial Registry

  https://www.mdpi.com/2072-6694/14/23/5935

  Ewing sarcoma (EwS) is a malignant bone and soft-tissue cancer that primarily affects adolescents and young adults. In rare cases, EwS develops as a secondary cancer; that is, after a previous cancer other than EwS. We collected information on all patients with EwS as a secondary cancer from three past international EwS studies to better understand affected patients and to identify potential at-risk patients. Forty-two patients with secondary EwS were identified, representing approximately 1.1% of all EwS cases. As primary cancers, patients suffered mainly from cancers of the blood-forming system, such as leukemia and lymphoma. We could not identify any risk factors for the development of EwS as a secondary cancer. Survival from a second cancer diagnosis with EwS is comparable to EwS as a first cancer diagnosis; therefore, patients with secondary EwS should also be offered complete therapy with the goal of cure, especially if the tumor is localized to only one site.

• #22 Ewing Sarcoma as Secondary Malignant Neoplasm—Epidemiological and Clinical Analysis of an International Trial Registry

  https://www.mdpi.com/2072-6694/14/23/5935

  Ewing sarcoma (EwS) is the second most common bone and soft tissue tumor, affecting primarily adolescents and young adults. Patients with secondary EwS are excluded from risk stratification in several studies and therefore do not benefit from new therapies. More knowledge about patients with EwS as secondary malignant neoplasms (SMN) is needed to identify at-risk patients and adapt follow-up strategies. Epidemiology, clinical characteristics, and survival analyses of EwS as SMN were analyzed in 3844 patients treated in the last three consecutive international EwS trials, EICESS 92, Euro-E.W.I.N.G. 99, and EWING 2008. Forty-two cases of EwS as SMN (approximately 1.1% of all patients) were reported, preceded by a heterogeneous group of malignancies, mainly acute lymphoblastic leukemias (n = 7) and lymphomas (n = 7).

• #23 Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis (Review)

  https://www.spandidos-publications.com/10.3892/ol.2021.12615

  The incidence of EES is 0.4 per million, which is 10 times less than that of ESB. Its prevalence follows a bimodal distribution, peaking in those who are 5 years and 35 years. Patients with EES tend to be older than those with ESB, and EES is not associated with sex or race unlike ESB. […] Imaging plays a central role in the diagnosis, staging, treatment monitoring and surveillance of EES. However, the imaging characteristics of EES are non-specific. EES can be diagnosed via ultrasonography (US), computed tomography (CT) or MR imaging, with each modality having its own specific indications. […] In addition to local staging of the primary tumor, imaging is also used to detect the presence of metastatic disease. CT scans of the chest are superior to conventional radiographs in detecting lung metastasis. […] Overall, definitive diagnosis is made with a CT-guided or ultrasound-guided core-needle biopsy, as well as pathological examination of the resected surgical specimen.

• #24 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  The presence or absence of metastatic disease is the single most powerful predictor of outcome. […] Approximately 25% of patients with Ewing sarcoma have metastatic disease at the time of diagnosis, with lung, bone, and bone marrow being the most common metastatic sites. […] The Surveillance, Epidemiology, and End Results (SEER) Program database was used to compare patients younger than 40 years with Ewing sarcoma who presented with skeletal and extraosseous primary sites. […] The time from the first symptom to diagnosis of Ewing sarcoma is often long, with a median interval reported from 2 to 5 months. […] The two major types of prognostic factors for patients with Ewing sarcoma are grouped as follows: Pretreatment factors and Response to initial therapy factors. […] The COG performed a retrospective analysis from two large cooperative trials that used similar treatment regimens.

• #25 Ewing Sarcoma: Presentation, Diagnosis, and Treatment | Consultant360

  https://www.consultant360.com/articles/ewing-sarcoma-presentation-diagnosis-and-treatment

  Ewing sarcoma is the second most common malignant bone tumor in children, after osteosarcoma. Peak incidence is in the second decade of life, with approximately 80% of patients being younger than 20. Ewing sarcoma has a slight male predominance with a ratio of 1.6 to 1.1. Its incidence is 2.93 per million people in the white population, and it is very rare in African and Asian populations. The shoulder region is the third most common site for bone and soft tissue tumors, which occur frequently in the proximal humerus, scapula, and clavicle. […] While Ewing sarcoma accounts for only 3% to 10% of all bone tumors, with the flat bones of the pelvis and long bones being the most frequent site, it is an aggressive tumor that may quickly metastasize. Nearly one-fourth of patients have detectable metastases at diagnosis; the lungs are the most common site, followed by bone and bone marrow. The 5-year survival rate for localized Ewing sarcoma is approximately 75%, with a much worse prognosis for persons with metastatic disease. […] Because Ewing sarcoma is such a rare disease, advances may only be possible with international trials and collaborative research. An improved understanding of Ewing sarcoma family of tumors likely will lead to enhanced diagnostic and staging technologies.

• #26 Current Status of Management and Outcome for Patients with Ewing Sarcoma

  https://www.mdpi.com/2072-6694/13/6/1202

  Ewing sarcoma is the second most common bone sarcoma after osteosarcoma with an annual incidence of 1–3 persons per million and a peak incidence in the second decade of life. There is a slight male predominance, and the incidence is much higher among Caucasians than among African Americans and Asians. Around 15% of Ewing sarcoma arises in the soft tissue, whereas 25% of bony Ewing sarcoma occurs in the pelvis and 20% occurs in the femur. If arising in the long bones, the tumor is typically located in the diaphysis. The lungs are the most common metastatic site (50%), followed by bone (25%). […] Ewing sarcoma is a highly aggressive malignancy affecting primarily children and adolescents. It is the second most common bone sarcoma among children, affecting between 1 and 3 persons per million inhabitants. The tumor typically carries a pathognomonic chromosomal translocation resulting in a fusion transcript (EWSR1-FLI1), which plays an orchestral role in tumor development. While the fusion transcript has been known for decades, targeted treatment has been disappointing. However, new molecular techniques such as next-generation sequencing have significantly increased our understanding of this rare disease.

• #27 Ewing Sarcoma Family of Tumors (ESFT) – An Introduction – Rein in Sarcoma

  https://www.reininsarcoma.org/ewing-sarcoma-an-introduction/

  Patients with primary pelvic tumors are significantly more likely to present with metastatic disease compared to other sites (25% vs. 16%). Other factors that may be associated with clinically evident metastatic disease at presentation include: High LDH, Fever, Interval between onset of symptoms and diagnosis 3 months, Age 12 years. […] The presence or absence of metastasis is the key prognostic factor for ESFT. Approximate five-year survival rates for patients with localized disease are 70%, while they average 33% for those who have overt metastasis at diagnosis. The location of metastasis also matters. Patients with bone and lung metastasis fare significantly worse than those with bone metastasis alone, who in turn, fared worse than those with isolated lung metastasis.

• #28 Bibliometric analysis of Ewing sarcoma from 1993 to 2022 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10723-7

  Ewing sarcoma has attracted more attention in recent years but has yet to be bibliometrically analyzed. […] The incidence rate accounts for 10 to 15% of orthopedic tumors. […] The peak incidence occurs at the age of 15, and the incidence rate in males is higher than in females, with a ratio of 3:2. […] The five-year overall survival rate of patients with both Ewing sarcoma and a localized disease is between 65 and 75%. […] The survival rate of patients is approximately 70% after five years and decreases sharply to 30% after 10 years. […] The poor survival rate of Ewing sarcoma has since gained awareness. […] The results revealed that the total number of Ewing sarcoma-related research papers published annually worldwide has gradually increased over the past three decades, reflecting its growing importance in the field of orthopedics.

• #29 Current Status of Management and Outcome for Patients with Ewing Sarcoma

  https://www.mdpi.com/2072-6694/13/6/1202

  Ewing sarcoma is the second most common bone sarcoma in children after osteosarcoma. It is a very aggressive malignancy for which systemic treatment has greatly improved outcome for patients with localized disease, who now see survival rates of over 70%. However, for the quarter of patients presenting with metastatic disease, survival is still dismal with less than 30% of patients surviving past 5 years. Patients with disease relapse, local or distant, face an even poorer prognosis with an event-free 5-year survival rate of only 10%. […] Management of patients with metastatic or treatment refractory Ewing is far from established since robust evidence is lacking. Several different polychemotherapy regimens have been used, largely dependent on institutional preferences and based on small studies. The investigators of the international multicenter trial on recurrent and primary refractory Ewing sarcoma (rEECur) have managed a great achievement by bringing together multiple centers in different countries in order to perform a randomized controlled trial to define the standard of care and best backbone chemotherapy regimen.

• #30 Complex Multidisciplinary Care of an AYA With Ewing Sarcoma

  https://www.oncnursingnews.com/view/complex-multidisciplinary-care-of-an-aya-with-ewing-sarcoma

  Ewing sarcoma is a highly aggressive bone and soft tissue cancer that is the second most common primary bone tumor for patients younger than 18 years. Approximately 50% of the Ewing sarcoma family of tumors occur in adolescent and young adults (AYAs), representing only 5% of all malignant diagnoses in the AYA population. This rare disease behaves aggressively, with a survival rate of approximately 75% for localized sarcoma and a 5-year survival rate of 20% to 30% for metastatic disease. […] This case report highlights the importance of multidisciplinary management in providing complex oncology care in AYAs with Ewing sarcoma. […] Throughout this patients treatment, he had immense social support from his family and our AYA social worker, along with emotional encouragement from the whole care team.

• #31

  https://link.springer.com/article/10.1007/s12094-024-03602-5

  Currently, most clinical guidelines recommend routine use of PET/CT for initial ES staging and surveillance. […] Systematic reviews on prognostic factors for ES 5-year overall survival (OS) conclude that independent prognostic factors on diagnosis are age (15 years vs 15 years), volume (200 mL vs 200 mL), primary tumor location (appendicular vs axial), and disease extent (local vs lung metastasis vs extra-pulmonary metastasis). […] Based on the above prognostic factors, the following risk groups have been proposed: standard-risk or good-risk (localized disease 200 mL and good histological response), High or poor risk (localized disease 200 mL or poor histological response). Patients with metastasis constitute the group with the poorest prognosis, and outcomes are better for lung metastasis than for extrapulmonary metastasis.

• #32 Ewing Sarcoma – TeachMePaediatrics

  https://teachmepaediatrics.com/haemonc/oncology/ewing-sarcoma/

  Ewing sarcoma is rare, with less than 100 cases diagnosed each year in the UK and more commonly affects males. The second decade of life, where bone growth is at its greatest, is the most common age for diagnosis. Ewing sarcoma can occur in adults, however, it is extremely rare in those older than 30 years. […] Prognostic factors associated with decreased survival rates include: Metastatic disease at diagnosis, Large tumour size (200ml in volume or 8cm in diameter), Primary tumour located in the axial skeleton, especially the pelvis, Histological response of less than 100%. […] Metastatic disease is a complication of Ewing sarcoma, with the most common sites for metastases being the lungs, other bones, and the bone marrow. Additionally, in around 30% of individuals, Ewing sarcoma recurs in later life and in these cases the disease is often fatal. Around 60% of individuals with Ewing sarcoma live at least 5 years after diagnosis.

• #33 A SEER-based nomogram accurately predicts prognosis in Ewing’s sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-021-02134-0

  Ewing’s sarcoma is a high-grade malignancy bone and soft tissue tumor that most commonly occurs in children and adolescents. […] The study aimed to determine the risk factors independently associated with the prognosis of Ewing’s sarcoma and to construct a nomogram to predict patient survival. […] Age, tumor size, primary site, N stage, and M stage are independent risk factors affecting the OS and CSS in Ewings sarcoma patients. Compared with the 7th TNM staging, the nomogram consisting of these factors was more accurate for risk assessment and survival prediction in patients with Ewings sarcoma, thus providing a novel reliable tool for risk assessment and survival prediction in Ewings sarcoma patients.

• #34 A tool for predicting overall survival in patients with Ewing sarcoma: a multicenter retrospective study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-09796-7

  The aim of this study was to establish and validate a clinical prediction model for assessing the risk of metastasis and patient survival in Ewing’s sarcoma (ES). […] Patients diagnosed with ES from the Surveillance, Epidemiology and End Results (SEER) database for the period 2010-2016 were extracted, and the data after exclusion of vacant terms was used as the training set (n=767). […] The results of multivariate cox regression showed that age, bone metastasis, tumor size, and chemotherapy were independent prognostic factors of ES patients. […] The 5-year overall survival (OS) rate for patients with localized ES has increased from approximately 10% to 55%-65%, taking advantage of the application of a combination of surgery, chemotherapy and radiotherapy. […] Limited by the low incidence of ES, researchers have difficulty recruiting a sufficient number of ES cases for their cohort studies.

• #35 A tool for predicting overall survival in patients with Ewing sarcoma: a multicenter retrospective study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-09796-7

  This study extracted clinicopathologic, and treatment-informed ES patient data from SEER data, subsequently used statistically validated variables to build and validate a clinical prediction model that could be used to predict OS at 1 and 3 years of ES patients. […] The model was also externally validated using data from up to 4 medical institutions. […] The tool built in this paper to predict 1- and 3-year survival in ES patients has a good identification and predictive power. […] The calibration plots showed that the model had a good agreement for patient survival at 1 and 3 years; ROC showed that it possessed a good predictive ability and clinical decision curve proved that it possessed good clinical utility. […] The model had a good clinical utility in improving both 1-year and 3-year survival in ES patients, with a slightly better net gain at 3 years than at 1 year.

• #36 A novel nomogram and risk classification system predicting the Ewing sarcoma: a population-based study | Scientific Reports

  https://www.nature.com/articles/s41598-022-11827-z

  Ewing sarcoma (ES) is a rare disease that lacks a prognostic prediction model. […] The clinicopathological data of ES were collected from the Surveillance, Epidemiology and Final Results (SEER) database from 2010 to 2018. […] The disease most often presents adjacent to bone, and a quarter arises in soft tissues. […] The median overall survival of ES patients is less than 12 months. […] Due to the progress of multimodal treatments such as surgery, chemotherapy and radiotherapy, the 5-year survival rate of local ES that responds to multimodal therapy has increased to 55-65%. […] In contrast, the survival rate of patients with metastases is less than 30% for 5 years. […] Due to the rarity of ES, its prognostic factors are still unclear, and there is no internationally recognized risk stratification scheme for ES patients.

• #37 A novel nomogram and risk classification system predicting the Ewing sarcoma: a population-based study | Scientific Reports

  https://www.nature.com/articles/s41598-022-11827-z

  A nomogram is a widely accepted prognostic model that integrates various prognostic factors to predict individual survival. […] The prognostic factors were determined through univariate and multiple Cox proportional hazard analyses. […] Based on these independent prognostic factors, a nomogram for OS was constructed. […] The nomogram provided an improved C-index compared with current studies and showed good discrimination and calibration. […] Based on the nomogram, ES patients were divided into three risk groups to predict their survival.

• #38 Real world data of Ewing sarcoma from a resource-limited setting with poor compliance to treatment leading to poor outcomes – ecancer

  https://ecancer.org/en/journal/article/1801-real-world-data-of-ewing-sarcoma-from-a-resource-limited-setting-with-poor-compliance-to-treatment-leading-to-poor-outcomes

  There is limited data from India on Ewing sarcoma (ES) patients. […] The incidence of ES in the United States was 3.93 per million during the period between 1973 and 2004. […] According to an Indian registry, bone cancers represented 0.9% of all cancers with ES as the most common bone cancer. […] The majority of patients present with advanced disease secondary to delayed diagnosis. […] Compliance to treatment is poor leading to poor survival outcomes. […] Our analysis presents real-world data from a resource-limited country where outcomes are significantly inferior and greatly influenced by delayed diagnosis, advanced presentation, social, financial and geographical barriers leading to poor compliance and poor outcomes to treatment. […] The poor outcomes in our study arise from various geographical, social and financial barriers that patients of LMICs face. […] This real world data from a resource-limited setting reports the delayed diagnosis of advanced disease and poor compliance to adjuvant consolidation chemotherapy in ES patients.

• #39 Ewing Sarcoma – Market Insight, Epidemiology, and Market Forecast – 2034

  https://www.marketresearch.com/DelveInsight-v4028/Ewing-Sarcoma-Insight-Epidemiology-Forecast-40683405/

  In 2023, the US accounted for the maximum share of the Ewing sarcoma Treatment Market in the 7MM, i.e., nearly 53%. […] The total incident cases of Ewing sarcoma in the 7MM comprised approximately 1,200 cases in 2023 and are projected to increase during the forecasted period. […] In the 7MM, the highest Ewing Sarcoma Incidence Cases were seen in the United States, accounting of ~41% of the total cases. […] In the US, among all the age groups, 10-14 years accounted for the highest number of Ewing sarcoma cases i.e., around 30%, followed by the age-group 15-19 years (26%), in 2023. […] As per the analysis, males are more likely to get Ewing Sarcoma than females. In the US, in 2023, ~300 incident cases of male Ewing Sarcoma were estimated.

• #40 Ewing sarcoma | Bone Cancer Research Trust

  https://www.bcrt.org.uk/information/information-by-type/ewing-sarcoma/

  Imaging such as x-rays and scans also help doctors to see the size of the tumour and exactly where in the body it is. […] Using all of the test results, doctors can confirm a diagnosis of Ewing sarcoma and decide on a treatment plan. […] You can find out more about clinical trials and which trials are open to Ewing sarcoma patients on our clinical trials webpage.

• #41 SciELO Brazil – Sarcoma de Ewing: epidemiologia e prognóstico dos pacientes tratados no Instituto de Oncologia Pediátrica, IOP-GRAACC-UNIFESP Sarcoma de Ewing: epidemiologia e prognóstico dos pacientes tratados no Instituto de Oncologia Pediátrica, IOP-G

  https://www.scielo.br/j/rbort/a/HNJGYZWgrB5VhfhBvnLv5sx/?lang=en

  The lungs are the commonest site for metastases (50%), and pulmonary metastases present better prognosis than observed for extrapulmonary metastases. […] Despite aggressive treatment, it is reported in the literature that around 20 to 40% of the patients with localized disease and 80% of the patients with metastases evolve to death due to disease recurrence or progression. […] The time that elapses until recurrence is a major prognostic factor, such that the survival rate from recurrences occurring more than two years after the initial diagnosis is 25%, but it is less than 10% among cases of early recurrence. […] Classically, Ewing’s tumor presents genetic abnormalities that can be identified in 80% of the cases, represented by the reciprocal translocation between chromosomes 11 and 22, i.e. t(11;22) (q24;q12), which results in expression of the protein EWS/FLI-1.

• #42 Ewing sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ewing-sarcoma/symptoms-causes/syc-20351071

  Ewing sarcoma mostly happens in children and young adults, although it can happen at any age. […] Major advances in the treatment of Ewing sarcoma have improved the outlook for this cancer. […] Healthcare professionals often suggest long-term monitoring for side effects after treatment. […] Ewing sarcoma is more common in people of European ancestry. […] There’s no way to prevent Ewing sarcoma. […] Ewing sarcoma can spread from where it started to other areas. Ewing sarcoma most often spreads to the lungs and to other bones. […] The strong treatments needed to control Ewing sarcoma can cause major side effects, both in the short and long term.

• #43 Current Status of Management and Outcome for Patients with Ewing Sarcoma

  https://www.mdpi.com/2072-6694/13/6/1202

  The risk of treatment-related subsequent primary neoplasms (SPNs) is an issue that has gained increased interest. Now that more childhood and young adulthood cancer patients survive and are starting to reach an age in which cancer is more common in the general population, we are observing an increasing number of SPNs. Although SPNs are seen after treatment of all childhood malignancies, Hodgkin’s lymphoma and Ewing sarcoma survivors belong to the group of patients that have shown the highest risks. […] Identifying high-risk patients is important in order not only to improve systemic treatment but also to optimize the choice of local treatment strategies. Extent and location of metastases, tumor size and site, patient age, national and institutional practice, and patient preference are all important factors affecting the choice of treatment and subsequently local control and overall survival.

• #44 Ewing sarcoma (brief information)

  https://www.gpoh.de/kinderkrebsinfo/content/diseases/solid_tumours/ewing_sarcoma/pohpatinfoewingkurz20101215/index_eng.html

  In the large paediatric treatment centres, children and adolescents with Ewing sarcoma receive therapy according to standardised treatment plans (protocols). These protocols are designed by experts and aim at steadily improving the patients survival rates while also reducing the risk of therapy-related late effects. Therapy according to such treatment protocols is usually carried out within therapy optimising trials or registries.

• #45 Bibliometric analysis of Ewing sarcoma from 1993 to 2022 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10723-7

  Of the 3016 articles, the USA contributed 35.9% of the research articles, demonstrating the strong collaboration and highest centrality with other countries. […] The most published journals of Ewing sarcoma are the top journals in the oncology field, including Cancer Research, Clinical Cancer Research, and Journal of Clinical Oncology. […] For keyword co-occurrence, the top 10 keywords were closely related to the age of epidemiology, prognosis, and treatment of Ewing sarcoma. […] Emergent keywords indicated emerging trends and research frontiers. […] The timeline of the cluster map of co-cited literature revealed that the treatment of Ewing sarcoma recently became a research hotspot.

• #46 Ewing Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/bone/hp/ewing-treatment-pdq

  The identification of the recurring t(11;22) translocation in most Ewing sarcoma tumors led to the discovery that most tumors classified as Ewing sarcoma had a translocation that juxtaposed a portion of the EWSR1 gene to a portion of a gene in the ETS family, resulting in a transforming transcript. […] It is now agreed that these tumors are sufficiently different from Ewing sarcoma and that they should be stratified and analyzed separately from Ewing sarcoma, even if they are treated with similar therapy.

• #47

  https://journals.lww.com/jaaos/Fulltext/2010/02000/Ewing_Sarcoma_Family_of_Tumors.4.aspx?generateEpub=Article%7Cjaaos:2010:02000:00004%7C%7C

  The Ewing sarcoma family of tumors (ESFT) consists of a group of tumors characterized by morphologically similar round-cell neoplasm and by the presence of a common chromosomal translocation. Although rare, such tumors constitute the third most frequent primary sarcoma of bone after osteosarcoma and chondrosarcoma. ESFT most commonly affects young children and adolescents. Because most patients with clinically apparent localized disease at diagnosis may also have occult metastatic (ie, systemic) disease, multidrug chemotherapy as well as local disease control with surgery and/or radiation therapy are indicated for all patients. Despite marked improvements in survival during the past 40 years for patients with localized disease, lesser improvements have been seen in patients with metastatic or recurrent disease. […] A better understanding of the complex biology of ESFT may lead to the successful development of biologically targeted therapies. As the regulatory pathways responsible for transformation, growth, and metastasis of ESFT become more refined, the number of potential therapeutic targets will expand.

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