This report details the CT and MR imaging findings of a primary orbital Ewing＇s sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mas...This report details the CT and MR imaging findings of a primary orbital Ewing＇s sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mass with needle-like bone reaction. On MRI, the mass showed homogeneous iso-intensity on T1WI, heterogeneous hyper- intensity on T2WI, and marked homogeneous enhancement. Meanwhile, the mass was hyper-intense on DWI, and ADC alue was 575x10-6 mm2/s. The mass showed as high rCBV and rCBF, prolonged MTT based on DCS- PWI, and wash-out pattern of TIC derived from the DCE-MRI. Our case suggests that functional MRI modalities, including DWI, DSC-PWI or DCE-MR, could provide additional information for differential diagnoses. Both CT and MRI should be performed and comprehensively analyzed for limiting differential diagnoses, determining lesion extension and facilitating operative approach.展开更多
Ewing’s sarcoma(EWS)is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults.Despite the efficacy of chemoradiotherapy in some cases,the cure rate for patients with metastatic and ...Ewing’s sarcoma(EWS)is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults.Despite the efficacy of chemoradiotherapy in some cases,the cure rate for patients with metastatic and recurrent disease remains low.Therefore,there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment.Epigenetic regulation,a crucial factor in physiological processes,plays a significant role in controlling cell proliferation,maintaining gene integrity,and regulating transcription.Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS.A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development.This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS,integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.展开更多
BACKGROUND Ewing’s sarcoma(ES)is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults.Primary renal ES is rare;only a few cases and a small case series have been do...BACKGROUND Ewing’s sarcoma(ES)is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults.Primary renal ES is rare;only a few cases and a small case series have been documented,and only four cases involved primary renal ES in older people(>65 years old).CASE SUMMARY Herein,we describe the radiological and pathological features of primary renal ES in an older person.A 76-year-old man complained of poor oral intake and was found to have a large cystic renal mass with indistinct margins on computed tomography.Ultrasound-guided biopsy revealed that the tumor contained small round blue cells.The patient underwent a right radical nephrectomy.The tumor cells showed diffuse membranous CD99,and nuclear friend leukemia integration 1 transcription factor and NK2 Homeobox 2.Fluorescence in situ hybridization revealed EWSR1 translocation.Postoperatively,18F-fluorodeoxyglucose positron emission tomography revealed no evidence of metastasis.The patient was diagnosed with primary renal ES.Six months following the surgery,local recurrence and distant metastasis were observed.Primary renal ES is rare and often lethal in older individuals.The specific imaging findings are unknown,and treatment protocols have not been standardized.CONCLUSION This case report describes the radiological and pathological features of primary renal ES in an older person.展开更多
Purpose: Bioinformatics-based approach to screen and analyze differentially expressed genes associated with the biological characteristics of Ewing sarcoma. Means: The GSE17674 dataset was selected for analysis, obtai...Purpose: Bioinformatics-based approach to screen and analyze differentially expressed genes associated with the biological characteristics of Ewing sarcoma. Means: The GSE17674 dataset was selected for analysis, obtained by data retrieval based on the GEO public database. The R language limma toolkit was used to screen DEmRNAs. After the data were normalized, the Metascape online analysis software and the R language clusterProfiler package were used to analyze the GO function and KEGG pathway enrichment of DEmRNAs lines, respectively. The string database was selected for PPI analysis, and the results were imported into Cytoscape software to derive the core modules and predicted core genes. The genes selected above were analyzed for tissue localization specificity. Results: Through the analysis of GSE17674, differentially expressed genes were screened out, and GO and KEGG analyses were performed on the differentially expressed genes. The GO functional enrichment analysis was mainly enriched in the process of muscle system, muscle contraction, myocyte development, contractile fibers, myogenic fibers, myofibers, myofibrillar segments, actin binding, structural composition of muscle, and actin filament binding. KEGG pathway analysis showed that the core pathways associated with the development of ES were the core genes for myocardial contraction, congestive cardiomyopathy, and hypertrophic cardiomyopathy. Five Hub genes were obtained based on Cytoscape prediction. Tissue localization specificity analysis of Hub genes was performed, and a total of 2 Hub genes with tissue specificity were screened;MYH6 was specifically expressed in cardiac cells and MYL1 was specifically expressed in skeletal muscle cells. Conclusions: The differential genes screened will help to understand the molecular mechanisms underlying the highly invasive and metastasis-prone biological characteristics of ES, as well as provide new ideas for clinical drug-targeted treatment of ES.展开更多
BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be ea...BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.展开更多
Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed durin...Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.展开更多
BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in ...BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.展开更多
Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Me...Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.展开更多
Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study,...Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.展开更多
Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES ...Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specifi c enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.展开更多
Primary extraosseous Ewing's sarcoma is a very rare clinical entity. In the report, we present the case of a 36year-old female patient who underwent resection of lung mass and was diagnosed as having Ewing's s...Primary extraosseous Ewing's sarcoma is a very rare clinical entity. In the report, we present the case of a 36year-old female patient who underwent resection of lung mass and was diagnosed as having Ewing's sarcoma by pathological examination. The clinical, imaging, and pathologic features of the case are described and discussed.展开更多
We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated ...We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated with neoadjuvant chemotherapy followed by wide excision of the patella and reconstruction of the extensor mechanism using split tendoachilles auto graft. The patella is an uncommon site for primary or metastatic tumors of the bone. ES, though rare, should be included in the differential diagnosis of swellings arising from the patella. Auto graft from the tendoachilles is a good alternative for reconstructing the extensor mechanism of the knee.展开更多
BACKGROUND Ewing’s sarcoma(ES)is regarded as a skeletal tumor,with few instances of extraskeletal ES.A primary ES in the ureter is extremely rare.CASE SUMMARY We report the case of a 69-year-old woman who presented w...BACKGROUND Ewing’s sarcoma(ES)is regarded as a skeletal tumor,with few instances of extraskeletal ES.A primary ES in the ureter is extremely rare.CASE SUMMARY We report the case of a 69-year-old woman who presented with intermittent flank pain and hematuria and was found to have a mass in the left ureter.Pathology of the excised mass indicated ES.The clinical treatment and pathologic characteristics in this case,and a review of the literature describing ES in the urinary system,are presented.CONCLUSION Due to the rarity and malignancy of ES in ureter,early diagnosis and prompt surgical treatment are critical.展开更多
Ewing’s sarcoma is a pediatric tumor that mainly occurs in soft tissues and bones. Malignant characteristics of Ewing’s sarcoma are correlated with expression of EWS oncogene. We achieved knockdown of EWS expression...Ewing’s sarcoma is a pediatric tumor that mainly occurs in soft tissues and bones. Malignant characteristics of Ewing’s sarcoma are correlated with expression of EWS oncogene. We achieved knockdown of EWS expression using a plasmid vector encoding EWS short hairpin RNA (shRNA) to increase anti-tumor mechanisms of taxifolin (TFL), a new flavonoid, in human Ewing’s sarcoma cells in culture and animal models. Immunofluorescence microscopy and flow cytometric analysis showed high expression of EWS in human Ewing’s sarcoma SK-N-MC and RD-ES cell lines. EWS shRNA plus TFL inhibited 80% cell viability and caused the highest decreases in EWS expression at mRNA and protein levels in both cell lines. Knockdown of EWS expression induced morphological features of differentiation. EWS shRNA plus TFL caused more alterations in molecular markers of differentiation than either agent alone. EWS shRNA plus TFL caused the highest decreases in cell migration with inhibition of survival, angiogenic and invasive factors. Knockdown of EWS expression was associated with removal of DNA methylation from p53 promoter, promoting expression of p53, Puma, and Noxa. EWS shRNA plus TFL induced the highest amounts of apoptosis with activation of extrinsic and intrinsic pathways in both cell lines in culture. EWS shRNA plus TFL also inhibited growth of Ewing’s sarcoma tumors in animal models due to inhibition of differentiation inhibitors and angiogenic and invasive factors and also induction of activation of caspase-3 for apoptosis. Collectively, knockdown of EWS expression increased various anti-tumor mechanisms of TFL in human Ewing’s sarcoma in cell culture and animal models.展开更多
Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy al...Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome,more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting.Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.展开更多
BACKGROUND Ewing sarcomas(ESs)are highly aggressive malignancy and are predominant in the long bones of extremities of children and young adults with a slight male predilection and rarely presents at extra skeletal lo...BACKGROUND Ewing sarcomas(ESs)are highly aggressive malignancy and are predominant in the long bones of extremities of children and young adults with a slight male predilection and rarely presents at extra skeletal locations.CASE SUMMARY A 55-year-old woman came to our hospital after finding elevated tumor biomarkers during her physical examination.Her enhanced computed tomography scan showed a jejunal mass.The patient underwent laparoscopic enterectomy.The mass was later diagnosed as ES,evidenced by fluorescence in situ hybridization whereby the GLP ES breakpoint region 1 probe was used,showing that more than 10%of the cells showed a red-green-yellow signal proving the breakpoint rearrangement of the ES breakpoint region 1 gene in chromosome 22.CONCLUSION We describe a case of localized ES at the jejunum in China based on the literature.展开更多
Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents,typically presenting between 10 and 20 years of age.Extraosseous sarcomas of the Ewing family in adults are rare.We report a ...Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents,typically presenting between 10 and 20 years of age.Extraosseous sarcomas of the Ewing family in adults are rare.We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach.Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses.Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.展开更多
The Clavicle is a rare primary site for Ewing sarcoma (ES). We report one case of patient with clavicular ES under our tracking and review the related literatures on management of this rare tumor. The patient was age ...The Clavicle is a rare primary site for Ewing sarcoma (ES). We report one case of patient with clavicular ES under our tracking and review the related literatures on management of this rare tumor. The patient was age of 35 years and were non metastaticat presentation. The patient received chemotherapy before and after the surgery. Claviculectomy was used. The patient had good functional outcomes with no pain of the shoulder at the follow-up of ten months. In conclusion, Ewing sarcoma of clavicle is a very rare tumor and different methods of surgical treatment can be used. Clavicle often can be resected without a need of reconstruction.展开更多
Background and aim of work: We aimed from this study to determine the response and local relapse free survival (RFS) of pediatric patients with localized Ewing’s sarcoma treated with accelerated hyper-fractionated RT...Background and aim of work: We aimed from this study to determine the response and local relapse free survival (RFS) of pediatric patients with localized Ewing’s sarcoma treated with accelerated hyper-fractionated RT. Patient and methods: This study was a nonrandomized uncontrolled phase II study and was conducted at clinical oncology department and south Egypt cancer institute;it involved 28 patients with histologically confirmed Ewing’s sarcoma;all of them were subjected to PET/CT whenever possible or MRI with contrast of the primary site, MSCT chest, bone scan, and LDH to ensure absence of metastasis followed by the protocol of accelerated hyper fractionated RT. Results: The overall response rate (ORR) was 92.9% by MRI with significant effect of the type of response on local RFS (P 0.002). The median local RFS of 28 patients with localized Ewing’s sarcoma was 30 ± 8.599 months with 95% CI = 13.147 - 46.853;the 3-year local control was 35%. Conclusion: Accelerated hyper fractionated RT didn’t achieve better results than standard fractionation RT, but it is recommended to be done on a large sample size, and multiple centers, and continued follow up is also recommended to evaluate 5-year LRFS, 5-year OS.展开更多
文摘This report details the CT and MR imaging findings of a primary orbital Ewing＇s sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mass with needle-like bone reaction. On MRI, the mass showed homogeneous iso-intensity on T1WI, heterogeneous hyper- intensity on T2WI, and marked homogeneous enhancement. Meanwhile, the mass was hyper-intense on DWI, and ADC alue was 575x10-6 mm2/s. The mass showed as high rCBV and rCBF, prolonged MTT based on DCS- PWI, and wash-out pattern of TIC derived from the DCE-MRI. Our case suggests that functional MRI modalities, including DWI, DSC-PWI or DCE-MR, could provide additional information for differential diagnoses. Both CT and MRI should be performed and comprehensively analyzed for limiting differential diagnoses, determining lesion extension and facilitating operative approach.
基金funded in part by the National Natural Science Foundation of China(No.82371877)Advanced Talents and Science and Technology Innovation Foundation at Yangzhou University(No.137011856,HS).
文摘Ewing’s sarcoma(EWS)is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults.Despite the efficacy of chemoradiotherapy in some cases,the cure rate for patients with metastatic and recurrent disease remains low.Therefore,there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment.Epigenetic regulation,a crucial factor in physiological processes,plays a significant role in controlling cell proliferation,maintaining gene integrity,and regulating transcription.Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS.A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development.This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS,integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.
文摘BACKGROUND Ewing’s sarcoma(ES)is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults.Primary renal ES is rare;only a few cases and a small case series have been documented,and only four cases involved primary renal ES in older people(>65 years old).CASE SUMMARY Herein,we describe the radiological and pathological features of primary renal ES in an older person.A 76-year-old man complained of poor oral intake and was found to have a large cystic renal mass with indistinct margins on computed tomography.Ultrasound-guided biopsy revealed that the tumor contained small round blue cells.The patient underwent a right radical nephrectomy.The tumor cells showed diffuse membranous CD99,and nuclear friend leukemia integration 1 transcription factor and NK2 Homeobox 2.Fluorescence in situ hybridization revealed EWSR1 translocation.Postoperatively,18F-fluorodeoxyglucose positron emission tomography revealed no evidence of metastasis.The patient was diagnosed with primary renal ES.Six months following the surgery,local recurrence and distant metastasis were observed.Primary renal ES is rare and often lethal in older individuals.The specific imaging findings are unknown,and treatment protocols have not been standardized.CONCLUSION This case report describes the radiological and pathological features of primary renal ES in an older person.
文摘Purpose: Bioinformatics-based approach to screen and analyze differentially expressed genes associated with the biological characteristics of Ewing sarcoma. Means: The GSE17674 dataset was selected for analysis, obtained by data retrieval based on the GEO public database. The R language limma toolkit was used to screen DEmRNAs. After the data were normalized, the Metascape online analysis software and the R language clusterProfiler package were used to analyze the GO function and KEGG pathway enrichment of DEmRNAs lines, respectively. The string database was selected for PPI analysis, and the results were imported into Cytoscape software to derive the core modules and predicted core genes. The genes selected above were analyzed for tissue localization specificity. Results: Through the analysis of GSE17674, differentially expressed genes were screened out, and GO and KEGG analyses were performed on the differentially expressed genes. The GO functional enrichment analysis was mainly enriched in the process of muscle system, muscle contraction, myocyte development, contractile fibers, myogenic fibers, myofibers, myofibrillar segments, actin binding, structural composition of muscle, and actin filament binding. KEGG pathway analysis showed that the core pathways associated with the development of ES were the core genes for myocardial contraction, congestive cardiomyopathy, and hypertrophic cardiomyopathy. Five Hub genes were obtained based on Cytoscape prediction. Tissue localization specificity analysis of Hub genes was performed, and a total of 2 Hub genes with tissue specificity were screened;MYH6 was specifically expressed in cardiac cells and MYL1 was specifically expressed in skeletal muscle cells. Conclusions: The differential genes screened will help to understand the molecular mechanisms underlying the highly invasive and metastasis-prone biological characteristics of ES, as well as provide new ideas for clinical drug-targeted treatment of ES.
基金Supported by the Science and Education Project of Neijiang First People’s Hospital。
文摘BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.
文摘Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.
文摘BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.
文摘Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.
文摘Background Primary Ewing＇s sarcoma/primitive neuroectodermal tumor （ES/PNET） of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.
文摘Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specifi c enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.
文摘Primary extraosseous Ewing's sarcoma is a very rare clinical entity. In the report, we present the case of a 36year-old female patient who underwent resection of lung mass and was diagnosed as having Ewing's sarcoma by pathological examination. The clinical, imaging, and pathologic features of the case are described and discussed.
文摘We report a case of Ewings sarcoma(ES) involving the patella in a young female. ES of patella is a rare entity. The patient was presented with anterior knee pain and swelling arising from the patella. She was treated with neoadjuvant chemotherapy followed by wide excision of the patella and reconstruction of the extensor mechanism using split tendoachilles auto graft. The patella is an uncommon site for primary or metastatic tumors of the bone. ES, though rare, should be included in the differential diagnosis of swellings arising from the patella. Auto graft from the tendoachilles is a good alternative for reconstructing the extensor mechanism of the knee.
文摘BACKGROUND Ewing’s sarcoma(ES)is regarded as a skeletal tumor,with few instances of extraskeletal ES.A primary ES in the ureter is extremely rare.CASE SUMMARY We report the case of a 69-year-old woman who presented with intermittent flank pain and hematuria and was found to have a mass in the left ureter.Pathology of the excised mass indicated ES.The clinical treatment and pathologic characteristics in this case,and a review of the literature describing ES in the urinary system,are presented.CONCLUSION Due to the rarity and malignancy of ES in ureter,early diagnosis and prompt surgical treatment are critical.
文摘Ewing’s sarcoma is a pediatric tumor that mainly occurs in soft tissues and bones. Malignant characteristics of Ewing’s sarcoma are correlated with expression of EWS oncogene. We achieved knockdown of EWS expression using a plasmid vector encoding EWS short hairpin RNA (shRNA) to increase anti-tumor mechanisms of taxifolin (TFL), a new flavonoid, in human Ewing’s sarcoma cells in culture and animal models. Immunofluorescence microscopy and flow cytometric analysis showed high expression of EWS in human Ewing’s sarcoma SK-N-MC and RD-ES cell lines. EWS shRNA plus TFL inhibited 80% cell viability and caused the highest decreases in EWS expression at mRNA and protein levels in both cell lines. Knockdown of EWS expression induced morphological features of differentiation. EWS shRNA plus TFL caused more alterations in molecular markers of differentiation than either agent alone. EWS shRNA plus TFL caused the highest decreases in cell migration with inhibition of survival, angiogenic and invasive factors. Knockdown of EWS expression was associated with removal of DNA methylation from p53 promoter, promoting expression of p53, Puma, and Noxa. EWS shRNA plus TFL induced the highest amounts of apoptosis with activation of extrinsic and intrinsic pathways in both cell lines in culture. EWS shRNA plus TFL also inhibited growth of Ewing’s sarcoma tumors in animal models due to inhibition of differentiation inhibitors and angiogenic and invasive factors and also induction of activation of caspase-3 for apoptosis. Collectively, knockdown of EWS expression increased various anti-tumor mechanisms of TFL in human Ewing’s sarcoma in cell culture and animal models.
文摘Ewing sarcoma family tumors(ESFT) are heter ogeneous,aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome,more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting.Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.
文摘BACKGROUND Ewing sarcomas(ESs)are highly aggressive malignancy and are predominant in the long bones of extremities of children and young adults with a slight male predilection and rarely presents at extra skeletal locations.CASE SUMMARY A 55-year-old woman came to our hospital after finding elevated tumor biomarkers during her physical examination.Her enhanced computed tomography scan showed a jejunal mass.The patient underwent laparoscopic enterectomy.The mass was later diagnosed as ES,evidenced by fluorescence in situ hybridization whereby the GLP ES breakpoint region 1 probe was used,showing that more than 10%of the cells showed a red-green-yellow signal proving the breakpoint rearrangement of the ES breakpoint region 1 gene in chromosome 22.CONCLUSION We describe a case of localized ES at the jejunum in China based on the literature.
文摘Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents,typically presenting between 10 and 20 years of age.Extraosseous sarcomas of the Ewing family in adults are rare.We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach.Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses.Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.
文摘The Clavicle is a rare primary site for Ewing sarcoma (ES). We report one case of patient with clavicular ES under our tracking and review the related literatures on management of this rare tumor. The patient was age of 35 years and were non metastaticat presentation. The patient received chemotherapy before and after the surgery. Claviculectomy was used. The patient had good functional outcomes with no pain of the shoulder at the follow-up of ten months. In conclusion, Ewing sarcoma of clavicle is a very rare tumor and different methods of surgical treatment can be used. Clavicle often can be resected without a need of reconstruction.
文摘Background and aim of work: We aimed from this study to determine the response and local relapse free survival (RFS) of pediatric patients with localized Ewing’s sarcoma treated with accelerated hyper-fractionated RT. Patient and methods: This study was a nonrandomized uncontrolled phase II study and was conducted at clinical oncology department and south Egypt cancer institute;it involved 28 patients with histologically confirmed Ewing’s sarcoma;all of them were subjected to PET/CT whenever possible or MRI with contrast of the primary site, MSCT chest, bone scan, and LDH to ensure absence of metastasis followed by the protocol of accelerated hyper fractionated RT. Results: The overall response rate (ORR) was 92.9% by MRI with significant effect of the type of response on local RFS (P 0.002). The median local RFS of 28 patients with localized Ewing’s sarcoma was 30 ± 8.599 months with 95% CI = 13.147 - 46.853;the 3-year local control was 35%. Conclusion: Accelerated hyper fractionated RT didn’t achieve better results than standard fractionation RT, but it is recommended to be done on a large sample size, and multiple centers, and continued follow up is also recommended to evaluate 5-year LRFS, 5-year OS.
