Ewing sarcoma (ES) is a deadly bone cancer that affects 10 percent to 15 percent of all young people and adolescents. It is a “classic” ES of bone, extra-skeletal ES, malignant small cell cancer of the chest wall (Askin disease), or soft tissue-based PNET (primitive neuroectodermal cancers). James Ewing initially reported it in 1921. These sarcomas share common immunohistochemistry, and histologic features, suggesting that they arise from distinct MSCs (mesenchymal stem cells).
Non-random chromosomal rearrangements that result in fused genes that encode abnormal transcription factors are what define ESFT (Ewing sarcoma family tumors). EWS-FLI-1 is formed as a result of the t (11;22) (q24; q12) rearrangement, which is connected to 85 percent of malignancies. The rest, 10 percent to 15 percent of instances, are caused by the t (21;12) (22;12) and other less frequent translocations, which result in the EWS-ERG union.
Although it can happen in practically any bone or soft tissue, the pelvic, femur and axial skeleton are the most often affected anatomical areas. Patients typically arrive in pain and with edema in the affected area. While the 5-year survival rates for the localized tumor have increased from less than 20 percent to more than 70 percent over the past forty years because of both multi-agent combination chemotherapy and local treatment, the risk of recurrence is still significant.
Most have localized disease, but almost all have subclinical metastatic cancer. A quarter of patients with initially confined illnesses eventually experience a recurrence. With overall survival of fewer than 30 percent in individuals with separated pulmonary metastases and much less than 20 percent in those who have bone and marrow interference, there is no established treatment for refractory and recurrent Ewing sarcoma. There is a great unmet need to create innovative therapeutics for ES, given the factors of toxicity and inadequate survival from metastatic illness.
Epidemiology
Less than 5 percent of all soft tissue tumors are Ewing sarcomas, the second leading primary bone malignancy in young people and adolescents with a mean age of fifteen years. In the US, there are more than 200 instances annually. Between 1973 to 2004, there were 2.93 new cases of Ewing sarcoma per million people in the US.
Around 30 percent of instances occur in children as young as 10, while another 30 percent occur in individuals who are older than 20, with the highest prevalence occurring between the ages of 10 and 15 years old.
A 3 in 1 male-to-female ratio indicates that men dominate. More White people are impacted than Asian, Black, African, or Hispanic people. This huge racial disparity has not yet been investigated. It is unknown how common Ewing sarcoma occurs among elderly people.
Anatomy
Pathophysiology
Ewing sarcoma’s cell of genesis is still not completely understood. Non-random genome alternations between the ETS (E-twenty-six or E26 transition) gene product and the EWS genome are the defining feature of ES. T (11; 22) (q24; q12) is the most prevalent gene translocation. In more than 80 percent of the incidence, the fusion of the FLI1 gene on 11q24 with the EWS gene on 22q12 results in the formation of the hybrid gene EWS-FLI1.
It is logical to infer that the resulting EWS-FLI1 mutated gene may contribute to the pathophysiology of ES since it behaves as an abnormal transcription cofactor. But the fundamental cause of the translocation is still not conclusively determined. The t(21; 22) (q22; q12)translocation, which is present in about 15 percent of instances and results in the union of the EWS and ERG genes on 21q22, is the second most frequent rearrangement of the EWS-ETS family of genes.
Other genetic changes reported in the literature include t (2;22) (q33; q12), t (7; 22) (p22; q12), and t (17;22) (q21; q12), wherein EWS is linked to the ETS genetic families FEV, ETV1, and E1AF. There have also been more complex gene refactorings and chromosomal rearrangements documented in the literature, while it is not yet known if this is linked to aggressive malignancy characteristics.
Etiology
There isn’t any conclusive evidence linking ES to environmental triggers, radiation exposure, drug use, or ancestry of cancer. Small retrospective, case-control research has been the only research conducted so far.
Genetics
Prognostic Factors
In ES, numerous prognostic variables have been found. Compared to cases with a lesion in the proximal extremities, those with ES in the distal extremities typically have a favorable prognosis. For the evaluation of the prognosis, the metastatic illness must be present.
Unilateral pulmonary engagement does appear to be associated with a favorable prognosis than bilateral pulmonary engagement, and individuals with solitary lung metastasis are more likely to have a favorable prognosis than individuals with extrapulmonary metastasis locations.
A bad prognosis is linked to the growth of large malignancies as well as bone and lung metastasis. Studies also showed that tumor size was a significant predictive factor. Aged less than fifteen years is a clinically meaningful prognostic marker, according to the data as a whole.
According to clinical research, patients who had undergone neoadjuvant chemotherapy and had little to no viable tumor remaining at the time of surgery did appear to do better than those who had a more live tumor. Additionally, a higher risk of relapse is linked to a poor response to adjuvant chemotherapy. EWSR1-ETS relocation is no longer regarded as a poor predictive factor; it should be highlighted.
Ewing sarcoma (ES) is a deadly bone cancer that affects 10 percent to 15 percent of all young people and adolescents. It is a “classic” ES of bone, extra-skeletal ES, malignant small cell cancer of the chest wall (Askin disease), or soft tissue-based PNET (primitive neuroectodermal cancers). James Ewing initially reported it in 1921. These sarcomas share common immunohistochemistry, and histologic features, suggesting that they arise from distinct MSCs (mesenchymal stem cells).
Non-random chromosomal rearrangements that result in fused genes that encode abnormal transcription factors are what define ESFT (Ewing sarcoma family tumors). EWS-FLI-1 is formed as a result of the t (11;22) (q24; q12) rearrangement, which is connected to 85 percent of malignancies. The rest, 10 percent to 15 percent of instances, are caused by the t (21;12) (22;12) and other less frequent translocations, which result in the EWS-ERG union.
Although it can happen in practically any bone or soft tissue, the pelvic, femur and axial skeleton are the most often affected anatomical areas. Patients typically arrive in pain and with edema in the affected area. While the 5-year survival rates for the localized tumor have increased from less than 20 percent to more than 70 percent over the past forty years because of both multi-agent combination chemotherapy and local treatment, the risk of recurrence is still significant.
Most have localized disease, but almost all have subclinical metastatic cancer. A quarter of patients with initially confined illnesses eventually experience a recurrence. With overall survival of fewer than 30 percent in individuals with separated pulmonary metastases and much less than 20 percent in those who have bone and marrow interference, there is no established treatment for refractory and recurrent Ewing sarcoma. There is a great unmet need to create innovative therapeutics for ES, given the factors of toxicity and inadequate survival from metastatic illness.
Less than 5 percent of all soft tissue tumors are Ewing sarcomas, the second leading primary bone malignancy in young people and adolescents with a mean age of fifteen years. In the US, there are more than 200 instances annually. Between 1973 to 2004, there were 2.93 new cases of Ewing sarcoma per million people in the US.
Around 30 percent of instances occur in children as young as 10, while another 30 percent occur in individuals who are older than 20, with the highest prevalence occurring between the ages of 10 and 15 years old.
A 3 in 1 male-to-female ratio indicates that men dominate. More White people are impacted than Asian, Black, African, or Hispanic people. This huge racial disparity has not yet been investigated. It is unknown how common Ewing sarcoma occurs among elderly people.
Ewing sarcoma’s cell of genesis is still not completely understood. Non-random genome alternations between the ETS (E-twenty-six or E26 transition) gene product and the EWS genome are the defining feature of ES. T (11; 22) (q24; q12) is the most prevalent gene translocation. In more than 80 percent of the incidence, the fusion of the FLI1 gene on 11q24 with the EWS gene on 22q12 results in the formation of the hybrid gene EWS-FLI1.
It is logical to infer that the resulting EWS-FLI1 mutated gene may contribute to the pathophysiology of ES since it behaves as an abnormal transcription cofactor. But the fundamental cause of the translocation is still not conclusively determined. The t(21; 22) (q22; q12)translocation, which is present in about 15 percent of instances and results in the union of the EWS and ERG genes on 21q22, is the second most frequent rearrangement of the EWS-ETS family of genes.
Other genetic changes reported in the literature include t (2;22) (q33; q12), t (7; 22) (p22; q12), and t (17;22) (q21; q12), wherein EWS is linked to the ETS genetic families FEV, ETV1, and E1AF. There have also been more complex gene refactorings and chromosomal rearrangements documented in the literature, while it is not yet known if this is linked to aggressive malignancy characteristics.
There isn’t any conclusive evidence linking ES to environmental triggers, radiation exposure, drug use, or ancestry of cancer. Small retrospective, case-control research has been the only research conducted so far.
In ES, numerous prognostic variables have been found. Compared to cases with a lesion in the proximal extremities, those with ES in the distal extremities typically have a favorable prognosis. For the evaluation of the prognosis, the metastatic illness must be present.
Unilateral pulmonary engagement does appear to be associated with a favorable prognosis than bilateral pulmonary engagement, and individuals with solitary lung metastasis are more likely to have a favorable prognosis than individuals with extrapulmonary metastasis locations.
A bad prognosis is linked to the growth of large malignancies as well as bone and lung metastasis. Studies also showed that tumor size was a significant predictive factor. Aged less than fifteen years is a clinically meaningful prognostic marker, according to the data as a whole.
According to clinical research, patients who had undergone neoadjuvant chemotherapy and had little to no viable tumor remaining at the time of surgery did appear to do better than those who had a more live tumor. Additionally, a higher risk of relapse is linked to a poor response to adjuvant chemotherapy. EWSR1-ETS relocation is no longer regarded as a poor predictive factor; it should be highlighted.
per day on day 1 every 21 days for 5 cycles in combination with vincristine and cyclophosphamide.
https://www.ncbi.nlm.nih.gov/books/NBK559183/
medtigo
Ewing sarcoma
Updated :
September 14, 2022
Ewing sarcoma (ES) is a deadly bone cancer that affects 10 percent to 15 percent of all young people and adolescents. It is a “classic” ES of bone, extra-skeletal ES, malignant small cell cancer of the chest wall (Askin disease), or soft tissue-based PNET (primitive neuroectodermal cancers). James Ewing initially reported it in 1921. These sarcomas share common immunohistochemistry, and histologic features, suggesting that they arise from distinct MSCs (mesenchymal stem cells).
Non-random chromosomal rearrangements that result in fused genes that encode abnormal transcription factors are what define ESFT (Ewing sarcoma family tumors). EWS-FLI-1 is formed as a result of the t (11;22) (q24; q12) rearrangement, which is connected to 85 percent of malignancies. The rest, 10 percent to 15 percent of instances, are caused by the t (21;12) (22;12) and other less frequent translocations, which result in the EWS-ERG union.
Although it can happen in practically any bone or soft tissue, the pelvic, femur and axial skeleton are the most often affected anatomical areas. Patients typically arrive in pain and with edema in the affected area. While the 5-year survival rates for the localized tumor have increased from less than 20 percent to more than 70 percent over the past forty years because of both multi-agent combination chemotherapy and local treatment, the risk of recurrence is still significant.
Most have localized disease, but almost all have subclinical metastatic cancer. A quarter of patients with initially confined illnesses eventually experience a recurrence. With overall survival of fewer than 30 percent in individuals with separated pulmonary metastases and much less than 20 percent in those who have bone and marrow interference, there is no established treatment for refractory and recurrent Ewing sarcoma. There is a great unmet need to create innovative therapeutics for ES, given the factors of toxicity and inadequate survival from metastatic illness.
Less than 5 percent of all soft tissue tumors are Ewing sarcomas, the second leading primary bone malignancy in young people and adolescents with a mean age of fifteen years. In the US, there are more than 200 instances annually. Between 1973 to 2004, there were 2.93 new cases of Ewing sarcoma per million people in the US.
Around 30 percent of instances occur in children as young as 10, while another 30 percent occur in individuals who are older than 20, with the highest prevalence occurring between the ages of 10 and 15 years old.
A 3 in 1 male-to-female ratio indicates that men dominate. More White people are impacted than Asian, Black, African, or Hispanic people. This huge racial disparity has not yet been investigated. It is unknown how common Ewing sarcoma occurs among elderly people.
Ewing sarcoma’s cell of genesis is still not completely understood. Non-random genome alternations between the ETS (E-twenty-six or E26 transition) gene product and the EWS genome are the defining feature of ES. T (11; 22) (q24; q12) is the most prevalent gene translocation. In more than 80 percent of the incidence, the fusion of the FLI1 gene on 11q24 with the EWS gene on 22q12 results in the formation of the hybrid gene EWS-FLI1.
It is logical to infer that the resulting EWS-FLI1 mutated gene may contribute to the pathophysiology of ES since it behaves as an abnormal transcription cofactor. But the fundamental cause of the translocation is still not conclusively determined. The t(21; 22) (q22; q12)translocation, which is present in about 15 percent of instances and results in the union of the EWS and ERG genes on 21q22, is the second most frequent rearrangement of the EWS-ETS family of genes.
Other genetic changes reported in the literature include t (2;22) (q33; q12), t (7; 22) (p22; q12), and t (17;22) (q21; q12), wherein EWS is linked to the ETS genetic families FEV, ETV1, and E1AF. There have also been more complex gene refactorings and chromosomal rearrangements documented in the literature, while it is not yet known if this is linked to aggressive malignancy characteristics.
There isn’t any conclusive evidence linking ES to environmental triggers, radiation exposure, drug use, or ancestry of cancer. Small retrospective, case-control research has been the only research conducted so far.
In ES, numerous prognostic variables have been found. Compared to cases with a lesion in the proximal extremities, those with ES in the distal extremities typically have a favorable prognosis. For the evaluation of the prognosis, the metastatic illness must be present.
Unilateral pulmonary engagement does appear to be associated with a favorable prognosis than bilateral pulmonary engagement, and individuals with solitary lung metastasis are more likely to have a favorable prognosis than individuals with extrapulmonary metastasis locations.
A bad prognosis is linked to the growth of large malignancies as well as bone and lung metastasis. Studies also showed that tumor size was a significant predictive factor. Aged less than fifteen years is a clinically meaningful prognostic marker, according to the data as a whole.
According to clinical research, patients who had undergone neoadjuvant chemotherapy and had little to no viable tumor remaining at the time of surgery did appear to do better than those who had a more live tumor. Additionally, a higher risk of relapse is linked to a poor response to adjuvant chemotherapy. EWSR1-ETS relocation is no longer regarded as a poor predictive factor; it should be highlighted.
https://www.ncbi.nlm.nih.gov/books/NBK559183/
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